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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
Q

When does lattice dystrophy onset?

A

Age 2 - 10

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26
Q

What are the signs and symptoms of lattice dystrophy?

A

VA reduction

RCE Common

Anterior stromal “inter-lacing” filamentous lesions

White spots

Central haze

27
Q

What is in this picture?

A

Lattice Type I White spots

28
Q
A

Lattice

29
Q

What does this picture depict?

A

Lattice refractile filamentary lines not to limbus

30
Q
A

Amber refractile material that may appear in lattice type 1 dystrophy

31
Q

What disease does lattice recur in?

A

Penetrating keratoplasty graft

32
Q

Whar differentiates lattice type I from type II?

A

Disimilar genotype

Central corneal sparring

33
Q

Whar other conditions are associated with lattice type II?

A

VII n palsy

Peripheral neuropathy

Amyloidosis

34
Q

What type of dystrophy is presented?

A

Granular

35
Q

What is the earliest dystrophy seen?

A

Granular

36
Q

What is rare in granular dystrophy?

A

RCE

37
Q

What type of dystrophy is associated with a unique area of italy?

A

Avellino which is similar to lattice and granular dystrophies

38
Q

How is macular dystrophy an exception to the dystrophy rule?

A

Starts in teens; marked in 20-30’s

Extends to periphery

39
Q

What dystrophy is the least common and also most severe?

A

Macular dystrophy

40
Q

What are some features of macular dystrophy in this photo?

A

Limbus to limbus, alll stromal depths

41
Q

How is Schnyder (crystalline) dystrophy an exception to the dystrophy rule?

A

Associated with a systemic disease (hypercholesterolemia)

42
Q

What are the features of Schnyder’s crystalline dystrophy?

A

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
Q

What are some features of Fleck Dystrophy?

A

Gray/white opacities

Odd shaped

Incidental finding

All levels of stroma

44
Q

What signs and symptoms are associated with Posterior Polymorphous Dystrophy (PPD)?

A

Asymtomatic; rare mild reduction in VA

Polymorphous opacities at the level of Decemet’s

Maybe corneal edema

45
Q

What percent of patients with PPD may develop increased IOP/glaucoma?

A

15%

46
Q

What endothelial dystrophies are represented by this photo?

A

Posterior Polymorphous Dystrophy (PPD)

47
Q

Identify the disease.

A

Posterior Polymorphous Dystrophy

48
Q

Why might PPD increase IOP and subsequently Glaucoma risk?

A

Endothelium grows across the trabeculum onto the iris and creates anterior synechia.

49
Q

How is Fuch’s Dystrophy an exception to the Dystrophy rule?

A

HIgher prevalence in postmenopausal females

May extend to the periphery

Multi-layer involvement

50
Q

Describe Fuch’s Dystrophy.

A

Progressive corneal disease in which corneal edema results from the primary metabolic incompetence of endothelial cells.

Endothelial barrier and pump failure

51
Q

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

A

Guttata

Stromal Edema

Epithelial Edema

52
Q

How should you confirm Fuch’s Dystrophy over just Guttata?

A

Using Pachymetry, if corneal edema is present like in Fuch’s then the cornea will be thicker.

53
Q

What are Hassal-Henle bodies?

A

Guttata in the periphery

54
Q

What is represented in this photo?

A

Guttata = excrescences of Descemet’s

55
Q

What disease is represented in this picture?

A

Guttata

56
Q
A

Fuch’s stromal and epithelial edema

57
Q
A

Fuch’s with clouding

58
Q
A

Edema

59
Q

How should you treat and manage Fuch’s?

A

Patient education

Lubricants

Hypertonics (5% gtt or ung)

RCE treatment protocol

Keratoplasty

60
Q

How many corneal transplants are done each year in the US and hwo many of those are as a result of Endothelial Dystrophies?

A

Approximately 38,000 with nearly half as a result of endothelial dystrophies.

61
Q

How does Congenital Hereditary Endothelial Dystrophy not follow the Dystrophy rules?

A

It is autosomal recessive

62
Q

What are the features of Congenital Hereditary Endothelial Dystrophy?

A

Present at birth of 1st decade

No guttata

Diffuse stromal edema

63
Q

Does congenital hereditary endothelial dystrophy respond to keratoplasty?

A

No, has poor results

Ocular Disease (7 decks)

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