Corneal Dystrophies Flashcards

1
Q

WIT: BM thickening with deposition of fibrillary protein between it and Bowman’s layer. Absence of hemidesmosomes on the basal epithelial cells.

A

EPMD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Pt presents w/ painful FBS due to bilateral erosions. Irregular astigmatism and monocular ghosting of images/diplopia.

A

EBMD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How do you treat EBMD?

A
  1. Muro 128
  2. Topical Lubricants/Ungs
  3. Epithelial Scraping
  4. Pressure Patch
  5. SCLs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Pt in his first few months of life presents with small, grey-white intraepithelial cysts in the intrapalpebral zone. On SLE, slightly thinned cornea, bleb-like lesions on retroillumination. Cornea is a regular size and shape.

A

Meesmann’s Dystrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Is vision affected in Meesmann’s Dystrophy? How do we treat it?

A

No, vision is often sparred.

If not, keratoplasty and CLs for RE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Meesmann’s dystrophy has what type of inheritance?

A

Autosomal dominant trait

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Pt in the 1st to 2nd decade of life presents with bilateral irregular epithelium with honeycomb pattern opacities in the region of Bowman’s layer with apparent scarring. RCEs are very common in this patient

A

Reis-Buckler’s Dystrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What’s the etiology of Reis-Buckler’s Dystrophy?

A
  1. Central Corneal Opacity

2. Fibrous tissue replaces bowman’s layer & epithelial BM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

In RB-Dystrophy, at age 5 what are the symptoms?

A
  1. RCEs
  2. Conj. injection
  3. Redness
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

In RB-Dystrophy, at age 30 what are the symptoms?

A
  1. RCEs are infrequent
  2. VA is affected
  3. Decreased corneal sensitivity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How do we treat RB-dystrophy?

A
  1. Lamellar Keratoplasty

2. Superficial Keratectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Pt presents with central hyaline opacities (milky) resembling crumbs, rings or snowflakes. Pattern looks like a Christmas tree. Pt complains of decreased vision and corneal sensation. Opacities never reach the limbus.

A

Granular Stromal Dystrophy: Type 1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the treatment for granular stromal dystrophy?

A

Penetrating keratoplasty by 5th decade

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Pt presents with refractile lines and white dot opacities. Pt has had RCEs in the past. Appears as a “ground glass appearance”. Stromal haze impairs his vision.

A

Lattice Stromal Dystrophy: type 1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

When does lattice stromal dystrophy appear? When does decreased vision occur?

A

late in the 1st decade

4th or 5th decade

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the tx for lattice stromal dystrophy?

A

Keratoplasty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

3 yo pt presents with decreased VA. SLE shows bilateral diffuse central stromal haze with poorly demarcated gray-white opacities throughout anterior stroma. No irritation or inflammation is present.

A

Macular Stromal Dystrophy

18
Q

Pt presents with bilateral donut-like or wreath-like opacities throughtout the stroma. Vision is unaffected. Noted with confocal microscopy.

A

Fleck Stromal Dystrophy

19
Q

Pt presents with these 6 types of bilateral opacities at stromal depth; dendritic, boomerang, circular, coma, linear, filiform. Vision is unaffected. Resembles cornea Farinata. WIT?

A

Primary Pre-Descemet Dystrophy

20
Q

Pt presents with dew drop like endothelial opacities, focal thickenenings on Descemet’s membrane. Located centrally.

A

Corneal guttata

21
Q

If corneal guttata is found in the peripheral cornea, what’s that called?

A

Hassal-henle bodies

22
Q

2’ gutatta results from what?

A
  1. corneal disease, trauma or inflammation
23
Q

Progressive corneal disease in which corneal edema results from faulty endothelial cells and pump fail.

A

Fuch’s endothelial dystrophy

24
Q

Fuch’s dystrophy has what type of inheritance?

A

Autosomal dominant

25
Q

Fuch’s dystrophy is associated with an increased prevalence of what disease?

A

POAG

26
Q

What are the 3 stages of Fuch’s dystrophy?

A
  1. Guttata
  2. Stromal Edema
  3. Epithelial Edema
27
Q

What stage of Fuch’s: appears as “ground glass” and folds in descemet’s. May see epithelial edema, vision can be affected. Hazy vision, severe pain if bullous keratopathy

A

Stage 2

28
Q

What stage of Fuch’s: endothelial pigment with moderate greying or thickening of descemet’s membrane, mainly asymptomatic

A

Stage 1

29
Q

What stage of Fuch’s: Subepithelial irregularities with grey, swirling sheets of scar tissue and stromal scarring. May not neo and bullous keratopathy. Hazy vision & glare, worse in AM

A

Stage 3

30
Q

How do you manage stage 1 of Fuch’s?

A

None, Muro

31
Q

How do you manage stage 2 of Fuch’s?

A
  1. Muro QID - 8x a day
  2. Hairdryer in AM
  3. SCLs
32
Q

How do you manage stage 3 of Fuch’s?

A
  1. Penetrating keratoplasty

2. Epithelial stripping

33
Q

Pt appears w/ bilateral central blisters, band-like or train tracks on cornea. Associated with iris membranes, PAS, ectropion uveitis, corectopia and glaucoma.

A

Posterior Polymorphous Dystrophy

34
Q

What is the etiology of PPD?

A
  1. Autosomal dominant
  2. Bilateral
  3. Congenital but not found until later in life
35
Q

What is tx for PPD?

A
  1. None (if good VA)
  2. penetrating keratoplasty
  3. DSEK
36
Q

Unilateral, abnormal corneal endothelium that causes Iris atrophy, 2’ angle closure glaucoma, PAS and corneal edema.

A

Iridocorneal Endothelial Syndrome (ICE Syndrome)

37
Q

What are the 3 clinical variations to ICE syndrome?

A
  1. Iris nevus
  2. Chandler’s Syndrome
  3. Essential iris atrophy
38
Q

Pt presents with pain, decreased vision, endothelial abnormality, PAS, iris atrophy, pigmented iris nodules, corneal edema and IOP spike.

A

ICE syndrome

39
Q

Cellular deposits on the corneal endothelium.

A

Corneal Keratic Precipitates

40
Q

Fresh KPs look ? Old KPs look ?

A
Fresh = white and round
Old = pigmented and irregular