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Ocular Disease I Part II > Corneal Dystrophy > Flashcards

Corneal Dystrophy Flashcards

(78 cards)

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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
What layers are affected by Reis-Buckler's Dystrophy and what impact does this dystrophy have on VA
Anterior stroma to Bowman's layer; VA decreases
26
What is the clinical presentation of Reis-Buckler's Dystrophy?
Bilateral and symmetrical w/ grey reticular opacities seen mainly in central cornea
27
ID the condition in the photo
Type I Bowman's membrane dystrophy aka Reis-Buckler's Dystrophy
28
ID the condition in the photos
Bowman's membrane layer dystrophy type II aka Thiel-Behnke honecomb
29
How does Lattice Dystrophy (Type I) present?
* anterior stromal “inter-lacing” filamentous lesions, white spots, central haze * Amyloid deposits * Rarely - get “lattice-like dystrophy” secondary to amyloidosis (milder and later onset)
30
What is the typical age of onset?
2-10 yrs of age
31
Name two charax of Lattice Dystrophy (Type I)
RCE is common and VA reduction occurs
32
ID the condition in the photo
Lattice Type I (Biber-Haab) 4 yo. White spots
33
Cracked glass appearance and the diffuse stromal haze are due to what in Lattice Dystrophy (Type I)
Lattice lines and edema respectively
34
PKP graft recurrence occurs more in \_\_\_ than in macular and granular Dystrophies
Lattice Type I
35
What three conditions are associated with Lattice Dystophy Type II?
* VII n palsy * peripheral neuropathy * amyloidosis
36
which geometric region of cornea is spared in Type II lattice dystrophy?
Central Corneal Sparing
37
Which of the dystrophies is the earliest to manifest?
Granular Corneal dystrophy In the first decade
38
What condition is presented in the photo?
Granular Corneal Dystrophy
39
How does Granular manifest
•Centrally discrete focal white deposits all at stromal depths, “cornflakes”, area between lesions is clear Unknown hyaline-like material
40
What are two features of Granular Corneal Dystrophy?
RCE is rare and VA is reduced and can be severe in patients older than 40
41
Name the type of dystrophy in the Photo
Avellino (Granular Type II)
42
Avellino shares the same gene loci as...
Lattice and Reis-Buckler's Dystrophies
43
Which is the most common and yet most severe of the dystrophies?
Macular Dystrophy
44
How does Macular dystrophy manifest?
•diffuse “ground-glass” haze lesions, corneal haze between lesions, gray-white or milky-white opacities throughout stroma, and limbus to limbus
45
The opacities seen in Macular Dystrophy are due to what?
Excess glycosaminoglycan. Abnormal keratocyte storage of mucopolysaccharide
46
List the distinct features of Macular Dystrophy
Autosomal recessive; extends to periphery; VA reduction starts in teens; marked in 20-30s; Photophobia; RCE is less than lattice dystrophy
47
What condition is presented in the photo?
Macular Dystrophy
48
What systemic condition is Schnyder's Central Crystalline associated with?
Systemic Hypercholestrolemia
49
How does Schnyder's Central Crystalline dystrophy manifest?
central crystals from annulus during 1st to 2nd decades, dense arcus ring froms during 3rd to 4th
50
What impact does Schnyder's Central Crystalline dystrophy have on VA? and is RCE involved
Can cause mild VA reduction (20/40) No RCE presents with this dystrophy
51
What condition is presented in the photo?
Schnyder's Crystalline dystrophy | (Rem S is for Sunflower)
52
Schnyder's is rare. T/F?
T
53
What condition is presented in the photo and what features are visible?
Schnyder's Central Crystalline Cholesterol deposits
54
What condition is presented in the photo?
Fleck Dystrophy
55
What are the manifestations of Fleck dystrophy?
gray/white opacities; odd shaped; inicidental finding all levels of stroma
56
How does Posterior Polymorphous Dystrophy (PPD) present?
Polymorphous opacities at the level of Descemet's
57
What are the symptoms of PPD
Can be asymptomatic rare reduction in VA about 20/30 Corneal edema may occur
58
What percentage of PPD patients may develop increased IOP?
15%
59
What condition is presented in the photo?
Posterior Polymorphous dystrophy
60
ID the condition in the photo
SL finding of Posterior polymorphous dystrophy
61
What are the features of Fuch's Dystrophy?
* Higher prevalence in females (postmenopausal) * Age-related (\>40) * May extend to periphery * Appears as multi-layer involvement
62
What are the symptoms of Fuch's Dystrophy?
* VA reduction in advancing stages * RCE
63
What are they objective clinical features of Fuch's Dystrophy?
* Guttata * Stromal edema * Epithelial edema
64
What are three considerations to be made in assessing Fuch's Dystrophy?
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
What is the pathophysiology of Fuch's Dystrophy?
* Na+K+ATPase pump and ion flow * Endothelial cells do not reproduce, but migrate
66
Hassal-Henle bodies refers to what?
Guttata in the periphery
67
Differentiate "normal" guttata from Fuch's
* guttata: slowly progressive; Seen in uveitis; transient * Fuchs’ dystrophy: increasing number of guttata accompanied by corneal edema
68
Describe normal guttata
normal endothelial aging; 11% of normals \>50yo;
69
What condition is presented in the photo?
Guttata
70
Identify the condition in the photo
Guttata
71
What condition is presented in the photos?
Fuch's Dystrophy
72
Describe Bullous Kerathopathy
severe stromal edema and corneal thickness \>30% à epithelial edema
73
What are the charax of stromal edema?
Hazy in appearance Possible folds in Descemet's
74
ID the condition in the photo
Fuch's stromal and epithelial edema
75
What are the treatment and management measures for Fuch's
* Patient education!! * Lubricants * Hypertonics (5% gtt or ung) * RCE treatment protocol * Keratoplasty-DLEK
76
How many PKPs are performed in the U.S. each year?
About 38,000
77
Endothelial dystrophies accounts for approx ____ of all visual loss that require PKPs to restore
half
78
What are the clinical manifestations and prognosis for Congenital Hereditary Endothelial Dystrophy?
* 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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