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Ocular Disease > Corneal Dystrophy > Flashcards

Corneal Dystrophy Flashcards

1
Q

What are the standard clinical characteristics of corneal dystrophies?

A

Autosomal Dominant

Onset by age 20

Bilateral

Slow progressive changes

No systemic diseases

No primary ocular diseases

Centrally located

Involves one single corneal layer

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2
Q

What is the one major exception to corneal dystrophies starting by age 20?

A

Fuch’s Endothelial Dystrophy

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3
Q

What is the most important characteristic of corneal dystrophies?

A

Primary involvement of single corneal layer

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4
Q

What is the most common corneal dystrophy?

A

Cogan Microcystic

“map-dot-fingerprint”

Epithelial Basement Membrane Dystrophy (EBMD)

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5
Q

How is EBMD considered an exception to the standard characteristics of corneal dystrophy?

A

It is non-hereditary

Typically shows up between 40 and 70

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6
Q

What is the risk of recurrent corneal erosion in EBMD?

A

10%

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7
Q

What does this picture represent?

A

Cogan Microcystic dystrophy or EBMD

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8
Q
A

EBMD

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9
Q

What type of lesion is this?

A

EBMD Map Lesion

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10
Q

What type of lesion is this?

A

EBMD Fingerprint lesion

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11
Q

What types of lesions are represented here?

A

Dots

Maps

Microcysts

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12
Q

What is the pathology of a dot lesion in EBMD?

A

Microcyst filled with cytoplasmic debris trapped by extra BM material

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13
Q

What disease is represented by this picture?

A

Recurrent Corneal Erosion

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14
Q

What is the treatment for EBMD?

A

Abrasion Protocol

Lubricants (ung at bedtime)

Hypertonics (Muro 128)

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15
Q

What is the abrasion protocol?

A

Bandage CL, pressure patch

Antibiotics, NSAID, cycloplegic

Doxycycline

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16
Q

What is a Meesmann?

A

Rare

Intraepithelial cysts because of abnormal basal cells and maturation to squamous and thick BM.

Identified as early as 6 months

Microcysts may rupture later in life

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17
Q

Symptoms of Meesmann?

A

Tearing

Photophobia

Vision Minimally Affected

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18
Q

What disease is this?

A

Meesmann

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19
Q

What is Reis-Bucklers Dystrophy?

A

When bowman’s layer is replaced by fibrocellular tissue, may also affect anterior stroma.

Rare

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20
Q

At what point in life does Reis-Bucklers Dystrophy occur?

A

Age 5-20, decreasing by age 30

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21
Q

What are the symptoms of Reis-Bucklers Dystropy?

A

Painful RCE

Irregular corneal surface

Scarring

Decreased VA

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22
Q

Reis-Bucklers Dystrophy

A

Reis Bucklers

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23
Q

When does Thiel-Behnke honeycomb Type II occur and what does it cause?

A

Later in the progression of Reis-Bucklers

Causes visual impairment

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24
Q

What may lattice dystrophy rarely be secondary to?

A

Amyloidosis

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25
When does lattice dystrophy onset?
Age 2 - 10
26
What are the signs and symptoms of lattice dystrophy?
VA reduction RCE Common Anterior stromal "inter-lacing" filamentous lesions White spots Central haze
27
What is in this picture?
Lattice Type I White spots
28
Lattice
29
What does this picture depict?
Lattice refractile filamentary lines not to limbus
30
Amber refractile material that may appear in lattice type 1 dystrophy
31
What disease does lattice recur in?
Penetrating keratoplasty graft
32
Whar differentiates lattice type I from type II?
Disimilar genotype Central corneal sparring
33
Whar other conditions are associated with lattice type II?
VII n palsy Peripheral neuropathy Amyloidosis
34
What type of dystrophy is presented?
Granular
35
What is the earliest dystrophy seen?
Granular
36
What is rare in granular dystrophy?
RCE
37
What type of dystrophy is associated with a unique area of italy?
Avellino which is similar to lattice and granular dystrophies
38
How is macular dystrophy an exception to the dystrophy rule?
Starts in teens; marked in 20-30's Extends to periphery
39
What dystrophy is the least common and also most severe?
Macular dystrophy
40
What are some features of macular dystrophy in this photo?
Limbus to limbus, alll stromal depths
41
How is Schnyder (crystalline) dystrophy an exception to the dystrophy rule?
Associated with a systemic disease (hypercholesterolemia)
42
What are the features of Schnyder's crystalline dystrophy?
Maybe mild VA reduction No RCE Central Haze from annulus ring during 1st and 2nd decades Dense arcus ring forms during 3rd and 4th decades
43
What are some features of Fleck Dystrophy?
Gray/white opacities Odd shaped Incidental finding All levels of stroma
44
What signs and symptoms are associated with Posterior Polymorphous Dystrophy (PPD)?
Asymtomatic; rare mild reduction in VA Polymorphous opacities at the level of Decemet's Maybe corneal edema
45
What percent of patients with PPD may develop increased IOP/glaucoma?
**_15%_**
46
What endothelial dystrophies are represented by this photo?
Posterior Polymorphous Dystrophy (PPD)
47
Identify the disease.
Posterior Polymorphous Dystrophy
48
Why might PPD increase IOP and subsequently Glaucoma risk?
Endothelium grows across the trabeculum onto the iris and creates anterior synechia.
49
How is Fuch's Dystrophy an exception to the Dystrophy rule?
HIgher prevalence in postmenopausal females May extend to the periphery Multi-layer involvement
50
Describe Fuch's Dystrophy.
Progressive corneal disease in which corneal edema results from the primary metabolic incompetence of endothelial cells. Endothelial barrier and pump failure
51
What are some objective clinical features of Fuch's Dystrophy?
Guttata Stromal Edema Epithelial Edema
52
How should you confirm Fuch's Dystrophy over just Guttata?
Using Pachymetry, if corneal edema is present like in Fuch's then the cornea will be thicker.
53
What are Hassal-Henle bodies?
Guttata in the periphery
54
What is represented in this photo?
Guttata = excrescences of Descemet's
55
What disease is represented in this picture?
Guttata
56
Fuch's stromal and epithelial edema
57
Fuch's with clouding
58
Edema
59
How should you treat and manage Fuch's?
Patient education Lubricants Hypertonics (5% gtt or ung) RCE treatment protocol Keratoplasty
60
How many corneal transplants are done each year in the US and hwo many of those are as a result of Endothelial Dystrophies?
Approximately 38,000 with nearly half as a result of endothelial dystrophies.
61
How does Congenital Hereditary Endothelial Dystrophy not follow the Dystrophy rules?
It is autosomal recessive
62
What are the features of Congenital Hereditary Endothelial Dystrophy?
Present at birth of 1st decade No guttata Diffuse stromal edema
63
Does congenital hereditary endothelial dystrophy respond to keratoplasty?
No, has poor results

Ocular Disease (7 decks)

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