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Ocular Disease I Part II > Corneal Dystrophy > Flashcards

Corneal Dystrophy Flashcards

1
Q

Define Dystrophy

A

Dystrophy is a developmental, symmetric and often hereditary changes occurring in original corneal tissue bcos of faulty nutrition, unrelated to other systemic or local diseases

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2
Q

What are the standard Clinical Charax of Corneal Dystrophies?

A

Autosomal Dominant; Usual onset of corneal findings 20; Bilateral; Slowly progressing changes; No systemic disease associated; No primary ocular disease Hx; Centrally located; Primary involvement of single corneal layer

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3
Q

What percentage of a family will show autosomal dominance for corneal dystrophies and what is the sex distribution?

A

50% or more and equal distribution between the sexes

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4
Q

Most corneal dystrophies show signs before 20. Which is the exception?

A

Fuch’s Dystrophy Usually seen in 30’s and 40’s and also more common in women

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5
Q

What is the MOST important clinical charax of corneal dystrophy?

A

Primary involvement of single corneal layer

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6
Q

What are the three anterior corneal dystrophies?

A

Coogan Microcystic; Meesman’s; Ries-Buckler’s corneal dystrophies

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7
Q

What are the six stromal corneal dystrophies?

A

Lattice; Granular; Macular; Schnyder’s; Fleck; Avellino (Type II Granular) corneal dystrophies

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8
Q

What are the two posterior corneal dystrophies?

A

Fuch’s and Posterior polymorphous corneal dystrophies

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9
Q

What is the most common corneal Dystrophy?

A

Cogan Microcystic or the Epithelium Basement Membrane Dystrophy (EBMD)

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10
Q

Name the condition in the photo and ID the features

A

Cogan Microcystic Dystrophy

A: Dots

B: Microcysts

C: Maps (most common)

D: Fingerprints (Least Common)

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11
Q

ID the condition in the photo

A

EBMD

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12
Q

ID the condition in the photo

A

Recurrent Corneal Erosion (RCE)

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13
Q

What is the treatment protocol for abrasion caused by EBMD

A

Bandage CL; Pressure patch; Antibiotics; NSAIDs; Cycloplegics; Doxycycline for MMP inhibition and anti-inflam

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14
Q

What are the three main management approaches for EBMD?

A

Lubrication with ung, abrasion reduction and hypertonics with 5% NaCl gtt, ung

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15
Q

What is a complication of RCE?

A

Corneal Scarring

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16
Q

What are the corneal manifestations of Meesmann Corneal Dystrophy?

A

Tiny intraepithelial round cysts identified as early as 6 months

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17
Q

What is the epidemiology of Meesmann Corneal Dystrophy?

A

Rare with unknown prevalence

First described in a specific German Population

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18
Q

What are two symptoms of Meesmann dystrophy?

A

Tearing and Photophobia

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19
Q

To what extent does Meesmann affect vision?

A

Minimally

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20
Q

The round microcysts of Meesmann Dystrophy can rupture. True/False

A

True.

They can rupture later in life

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21
Q

ID the condition in the photos

A

Meesmann Corneal dystrophy

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22
Q

ID the condition in the photos

A

Meesmann Corneal Dystrophy

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23
Q

What are the corneal manifestations of Reis-Buckler’s Dystrophy?

A

Painful RCE

Irregular Corneal Surface

Corneal Scarring

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24
Q

In Reis-Buckler’s Dystrophy, when are painful RCE occuring and by what age do they decrease?

A

5-20 yrs old with decreasing episodes by 30

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25
Q

What layers are affected by Reis-Buckler’s Dystrophy and what impact does this dystrophy have on VA

A

Anterior stroma to Bowman’s layer;

VA decreases

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26
Q

What is the clinical presentation of Reis-Buckler’s Dystrophy?

A

Bilateral and symmetrical w/ grey reticular opacities seen mainly in central cornea

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27
Q

ID the condition in the photo

A

Type I Bowman’s membrane dystrophy aka Reis-Buckler’s Dystrophy

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28
Q

ID the condition in the photos

A

Bowman’s membrane layer dystrophy type II aka Thiel-Behnke honecomb

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29
Q

How does Lattice Dystrophy (Type I) present?

A
  • anterior stromal “inter-lacing” filamentous lesions, white spots, central haze
  • Amyloid deposits
  • Rarely - get “lattice-like dystrophy” secondary to amyloidosis (milder and later onset)
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30
Q

What is the typical age of onset?

A

2-10 yrs of age

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31
Q

Name two charax of Lattice Dystrophy (Type I)

A

RCE is common and VA reduction occurs

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32
Q

ID the condition in the photo

A

Lattice Type I (Biber-Haab)
4 yo. White spots

33
Q

Cracked glass appearance and the diffuse stromal haze are due to what in Lattice Dystrophy (Type I)

A

Lattice lines and edema respectively

34
Q

PKP graft recurrence occurs more in ___ than in macular and granular Dystrophies

A

Lattice Type I

35
Q

What three conditions are associated with Lattice Dystophy Type II?

A
  • VII n palsy
  • peripheral neuropathy
  • amyloidosis
36
Q

which geometric region of cornea is spared in Type II lattice dystrophy?

A

Central Corneal Sparing

37
Q

Which of the dystrophies is the earliest to manifest?

A

Granular Corneal dystrophy

In the first decade

38
Q

What condition is presented in the photo?

A

Granular Corneal Dystrophy

39
Q

How does Granular manifest

A

•Centrally discrete focal white deposits all at stromal depths, “cornflakes”, area between lesions is clear
Unknown hyaline-like material

40
Q

What are two features of Granular Corneal Dystrophy?

A

RCE is rare and VA is reduced and can be severe in patients older than 40

41
Q

Name the type of dystrophy in the Photo

A

Avellino (Granular Type II)

42
Q

Avellino shares the same gene loci as…

A

Lattice and Reis-Buckler’s Dystrophies

43
Q

Which is the most common and yet most severe of the dystrophies?

A

Macular Dystrophy

44
Q

How does Macular dystrophy manifest?

A

•diffuse “ground-glass” haze lesions, corneal haze between lesions, gray-white or milky-white opacities throughout stroma, and limbus to limbus

45
Q

The opacities seen in Macular Dystrophy are due to what?

A

Excess glycosaminoglycan. Abnormal keratocyte storage of mucopolysaccharide

46
Q

List the distinct features of Macular Dystrophy

A

Autosomal recessive; extends to periphery; VA reduction starts in teens; marked in 20-30s; Photophobia; RCE is less than lattice dystrophy

47
Q

What condition is presented in the photo?

A

Macular Dystrophy

48
Q

What systemic condition is Schnyder’s Central Crystalline associated with?

A

Systemic Hypercholestrolemia

49
Q

How does Schnyder’s Central Crystalline dystrophy manifest?

A

central crystals from annulus during 1st to 2nd decades, dense arcus ring froms during 3rd to 4th

50
Q

What impact does Schnyder’s Central Crystalline dystrophy have on VA? and is RCE involved

A

Can cause mild VA reduction (20/40)

No RCE presents with this dystrophy

51
Q

What condition is presented in the photo?

A

Schnyder’s Crystalline dystrophy

(Rem S is for Sunflower)

52
Q

Schnyder’s is rare. T/F?

A

T

53
Q

What condition is presented in the photo and what features are visible?

A

Schnyder’s Central Crystalline

Cholesterol deposits

54
Q

What condition is presented in the photo?

A

Fleck Dystrophy

55
Q

What are the manifestations of Fleck dystrophy?

A

gray/white opacities; odd shaped; inicidental finding
all levels of stroma

56
Q

How does Posterior Polymorphous Dystrophy (PPD) present?

A

Polymorphous opacities at the level of Descemet’s

57
Q

What are the symptoms of PPD

A

Can be asymptomatic

rare reduction in VA about 20/30

Corneal edema may occur

58
Q

What percentage of PPD patients may develop increased IOP?

A

15%

59
Q

What condition is presented in the photo?

A

Posterior Polymorphous dystrophy

60
Q

ID the condition in the photo

A

SL finding of Posterior polymorphous dystrophy

61
Q

What are the features of Fuch’s Dystrophy?

A
  • Higher prevalence in females (postmenopausal)
  • Age-related (>40)
  • May extend to periphery
  • Appears as multi-layer involvement
62
Q

What are the symptoms of Fuch’s Dystrophy?

A
  • VA reduction in advancing stages
  • RCE
63
Q

What are they objective clinical features of Fuch’s Dystrophy?

A
  • Guttata
  • Stromal edema
  • Epithelial edema
64
Q

What are three considerations to be made in assessing Fuch’s Dystrophy?

A

Differential Dx with guttata
•Stromal and epithelial edema occur with breakdown of the endothelial barrier and pump
•Pachymetry: may help assess degree of corneal thickness (stromal edema)

65
Q

What is the pathophysiology of Fuch’s Dystrophy?

A
  • Na+K+ATPase pump and ion flow
  • Endothelial cells do not reproduce, but migrate
66
Q

Hassal-Henle bodies refers to what?

A

Guttata in the periphery

67
Q

Differentiate “normal” guttata from Fuch’s

A
  • guttata: slowly progressive; Seen in uveitis; transient
  • Fuchs’ dystrophy: increasing number of guttata accompanied by corneal edema
68
Q

Describe normal guttata

A

normal endothelial aging; 11% of normals >50yo;

69
Q

What condition is presented in the photo?

A

Guttata

70
Q

Identify the condition in the photo

A

Guttata

71
Q

What condition is presented in the photos?

A

Fuch’s Dystrophy

72
Q

Describe Bullous Kerathopathy

A

severe stromal edema and corneal thickness >30% à epithelial edema

73
Q

What are the charax of stromal edema?

A

Hazy in appearance

Possible folds in Descemet’s

74
Q

ID the condition in the photo

A

Fuch’s stromal and epithelial edema

75
Q

What are the treatment and management measures for Fuch’s

A
  • Patient education!!
  • Lubricants
  • Hypertonics (5% gtt or ung)
  • RCE treatment protocol
  • Keratoplasty-DLEK
76
Q

How many PKPs are performed in the U.S. each year?

A

About 38,000

77
Q

Endothelial dystrophies accounts for approx ____ of all visual loss that require PKPs to restore

A

half

78
Q

What are the clinical manifestations and prognosis for Congenital Hereditary Endothelial Dystrophy?

A
  • Not typical dystrophy: AR
  • Present at birth or 1st decade
  • No guttata
  • Diffuse stromal edema
  • Tx poor results with keratoplasty

Ocular Disease I Part II (2 decks)

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