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OD2 - Applied Clinical Training & Research Studies in Vision and Optometry > AED - Corneal Dystrophies - Week 10 > Flashcards

AED - Corneal Dystrophies - Week 10 Flashcards

1
Q

Are corneal dystrophies normally uni- or bilateral?

A

Bilateral

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2
Q

Are corneal dystrophies fast or sloww progressing?

A

Slowly progressive

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3
Q

What region of the cornea is typically affected by corneal dystrophy?

A

Central region

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4
Q

Are corneal dystrophies inflammatory?

A

Non-inflammatory

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5
Q

Do corneal dystrophies have associated ocular or systemic disease?

A

No

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6
Q

What is the usual onset for corneal dystrophy?

A

2nd decade

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7
Q

How many layers do corneal dystrophies primarily involve?

A

Primarily involves a single corneal layer

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8
Q

List two dystrophies of the corneal epithelium.

A

Epithelial basement membrane dystrophy

Meesmanns dystrophy

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9
Q

List a dystrophy of bowmans layer.

A

Reis-bucklers dystrophy

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10
Q

List four dystrophies of the corneal stroma.

A

Lattice dystrophy
Granular dystrophy
Macular dystrophy
Schnyders crystalline dystrophy

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11
Q

List two dystrophies of the corneal endothelium.

A

Fuchs dystrophy

Posterior polymorphous dystrophy

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12
Q

Is it easy to classify corneal dystrophies? Explain (2). What complicates this process (2)?

A

It is difficult dut to their relative rarity and heterogenous clinical appearance
Complicated by studies showing ‘distinct’ dystrophies are mutations in the same gene
Also individual dystrophies encompass mutations on completely different genes

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13
Q

What is the most common of all corneal dystrophies? Why is it often misdiagnosed?

A

Epithelial basement membrane dystrophy

Often misadiagnosed because of its variable appearance

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14
Q

Describe the inheritance of epithelial basement membrane dystrophy (2).

A

Many cases have no inheritance documented

Some autosomal dominant familial cases

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15
Q

What is the onset of epithelial basement membrane dystrophy? Is it common in children?

A

Usually adulthood

Rarely observed in children

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16
Q

What is the pathology of epithelial basement membrane dystrophy (list the cause and what this results in)

A

Abnormal maturation, production, and turnover of the basement membrane, leading to poor epithelial attachment.

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17
Q

Describe the signs of epithelial basement membrane dystrophy collectively known as ‘maps’ (shape, colour, appearance, edge).

A

Irregular islands of thickened, grey, hazy epithelium with scalloped edges

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18
Q

Describe the signs of epithelial basement membrane dystrophy collectively known as ‘dots’ (shape, colour, appearance).

A

Irregular, round or comma-shaped, non-staining, grey opacities

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19
Q

Describe the signs of epithelial basement membrane dystrophy collectively known as ‘fingerprints’. What slit lamp thechnique is best for viewing this?

A

Parallel, curvilinear lines, paracentral

Best seen with retroillumination

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20
Q

What does negative staining indicate, and how does it relate to epithelial basement membrane dystrophy?

A

Areas of elevated epithelium

Is a sign of epithelial basement membrane dystrophy

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21
Q

List 4 symptoms of epithelial basement membrane dystrophy. What causes these to manifest?

A 
Asymptomatic but can have periodic:
Mild blurred visiond
Image ghosting
Irritation
Occurs as cysts rupture
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22
Q

Consider the cause of symptoms of epithelial basement membrane dystrophy. What can occur in ~10% of patients and what does the risk of this increase with? List 5 symptoms of this.

A 
Recurrent corneal erosion, increasing incidence with age
Pain
FB sensation with specific onset
Potatophobia
Decreased vision
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23
Q

List 2 signs of epithelial basement membrane dystrophy.

A

Epithelial microcysts can rupture from time to time

Results in SPK and irritation

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24
Q

What is the assessment for epithelial basement membrane dystrophy (3)?

A

History and careful slit lamp with fluorescein

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25
Q

What is the management for epithelial basement membrane dystrophy (3)?

A

Lubricants
Management of co-existing surface diseases if symptomatic
Treat RCE if required

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26
Q

Is meesmanns dystrophy common or rare?

A

Very rare

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27
Q

What is the mode of inheritance for meesmans dystrophy?

A

Autosomal dominant

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28
Q

What is the onset for meesmanns dystrophy? Is it rapid or slow progressing?

A

Early childhood and slowly progressive

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29
Q

At what age do symptoms of meesmans dystrophy typically manifest?

A

Fourth decade

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30
Q

What is the pathology of meesmanns dystrophy?

A

Due to a mutation in keratin genes

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31
Q

List 4 symptoms of meesmans dystrophy.

A

Transient blurred vision
Surface irritation
Mild FB sensation
Photophobia

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32
Q

List a sign of meesmanns dystrophy (number, size, layer, region).

A

Multiple, tiny intra-epithelial vesicles (cysts) extending to the limbus

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33
Q

Where are lesions of meesmans dystrophy most common? What slit lamp technique is best to visualise them?

A

Most numerous in the intra-palpebral area

Best seen in retro-illumination

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34
Q

What is the treatment for meesmanns dystrophy (2)?

A

Surface lubrication

Bandage contact lenses

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35
Q

What layer does reis-bucklers dystrophy affect and what is this layer replaced with? Is it common or rare?

A

Affects bowmans layer

Replaced by a sheet-like connective tissue layer

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36
Q

What is the mode of inheritance for reis-bucklers dystrophy?

A

Autosomal dominant

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37
Q

What is the onset for reis-bucklers dystrophy?

A

Childhood

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38
Q

List 5 symptoms of reis-bucklers dystrophy.

A 
Red/sore eyes
Photophobia
Watery eyes
Ocular surface discomfort
Increasing blurred vision with age
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39
Q

What happens to vision over time with reis-bucklers dystrophy? Explain why.

A

Blurs

The modified bowmans layer thickens over time

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40
Q

What type collagen does bowmans layer normally have and how are fibres arranged? What happens with reis-bucklers dystrophy?

A

Type I collagen arranged randomly with pores for nerves

In reis-bucklers dystrophy, the layer is obliterated and replaced by randomly arranged collagen that thickens over time

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41
Q

List a sign of reis-bucklers dystrophy.

A

Slowly progressive deterioration of vision

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42
Q

How does reis-bucklers dystrophy appear early on? What about later into the disease?

A

Early childhood - fine, patchy supepithelial opacities

Late stage - polycystic pattern extending to the mid periphery

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43
Q

What is the management for reis-bucklers dystrophy (2)?

A

Manage RCEs as required

Monitor for decreased vision

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44
Q

Around what time is surgical intervention necessary for reis-bucklers dystrophy? List two surgery options.

A

Usually needed by the fifth decade
Refer for superficial keratectomy or PTK
May require keratoplasty

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45
Q

Is it common for reis-bucklers dystrophy to recur following surgical intervention?

A

Yesd

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46
Q

Is thiel-behnke corneal dystrophy common or rare? What is the mode of inheritance?

A

Very rare

Autosomal dominant

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47
Q

What is the onset for thiel-behnke corneal dystrophy?

A

Late childhood

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48
Q

List 4 symptoms of thiel-behnke corneal dystrophy. What other dystrophy is it similar to? How can they be distinguished by symptoms?

A

Red/sore eyes
Photophobia
Ocular surface discomfort
Increasing blurred bision with age
Very similar (possible variant) of reis-bucklers dystrophy
Blurred vision occurs later than in reis-bucklers dystrophy

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49
Q

List a sign of thiel-behnke corneal dystrophy.

A

Symmetrical, sub-epithelial, reticular opacities (honeycomb-like)

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50
Q

What is the management for thiel-behnke corneal dystrophy?

A

As per reid-bucklers dystrophy

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51
Q

What is the most common stromal dystrophy?

A

Lattice dystrophy

52
Q

What is lattice dystrophy caused by?

A

Amyloid deposits at different levels within the stroma

53
Q

What is the mode of inheritance for lattice dystrophy? How many variants of lattice dystrophy is there?

A

Autosomal dominant

4 variants

54
Q

What is the onset of lattice dystrophy?

A

Variable

55
Q

List two signs of lattice dystrophy.

A

Decreased corneal sensation

Reduced visual acuity over time

56
Q

What does lattice dystrophy look like?

A

Lattice-like corneal folds within the cornea - similar to gonio folds, but lattice pattern

57
Q

List 5 symptoms of lattice dystrophy.

A 
Ocular discomfort
Photophobia
Blurred vision
RCE
Visual impairment over time
58
Q

Do all variants of lattice dystrophy share the same mode of inheritance?

A

Yesd autosomal dominant

59
Q

Distinguish type II lattice dystrophy from type I (3).

A

Lattice lines are thicker, but less numerous (in type II)
Lines begin peripherally and extend centrally
Later onset vs type I

60
Q

What is a differential diagnosis for lattice dystrophy?

A

Polymorphic amyloid degneration

61
Q

What is the treatment for lattice degeneration (2)?

A

Monitor closely and manage RCE/discomfort

Refer if vision is significantly reduced for PTK

62
Q

What does success of surgical intervention for lattice dystrophy depend on? What may some forms require? Is recurrence possible? Compare to reis-bucklers dystrophy.

A

Depends on the depth of lines
Some forms may require lamellar graft or penetrating keratoplasty
Recurrence of dystrophy possible, but takes longer than reis-bucklers dystrophy

63
Q

Is granular dystrophy common or rare?

A

very rare

64
Q

What is granular dystrophy caused by? What is the mode of inheritance and onset?

A

Caused by hyaline deposits at different levels within the stroma
Autosomal dominant
Childhood onset

65
Q

What region of the eye does granular dystrophy appear initially and what does it look like? What can happen to them and what happens to them eventually (layer)?

A

Initially central, with a crumb-like opaque appearance
Can coalesce
Eventually involves deeper stroma

66
Q

What does the area between lesions of granular dystrophy have the appearance of?

A

Ground glass

67
Q

List a differential diagnosis for granular dystrophy and give two examples.

A

Other anterior stromal dystrophies

  • reis-bucklers dystrophy
  • macular dystrophy
68
Q

What is the treatment for granular dystrophy (4)?

A

Early on - lubricants

With time, bandage contact lenses, superficial keratecomy/PTK and keratoplasty

69
Q

What two dystrophies is avellino dystrophy a variant of? What is it characterised by (layer and lattice appearance)?

A

Variant of granular and lattice dystrophies

Characterised by granular deposits in the anterior stroma and deeper lattice lines

70
Q

What is the cause of avellino dystrophy (2)?

A

Deposition of both amyloid and hyaline in the corneal stroma

71
Q

Who can many patients with avellino dystrophy trace their ancestry back to? Is this clinical profile exclusive to them?

A

Avellino in italy, but clinical profile may not be exclusive

72
Q

Is avellino dystrophy common or rare? what is the mode of inheritance and what onset?

A

Rare
Autosomal dominant
Usually 1st or 2nd decades

73
Q

How does avellino dystrophy appear (general shapes)?

A

Icicle.star-shaped stromal opacities among disk-shaped opacities

74
Q

List 3 symptoms of avellino dystrophy.

A

Pain
Recurrent corneal erosion
Reduced vision

75
Q

Give a differential diagnosis for avellino dystrophy. Give three examples.

A

Other anterior stromal dystrophies
Reis-bucklers dystrophy
Granular dystrophy
Lattice dystrophy

76
Q

What is the treatment for avellino dystrophy?

A

As for other stromal dystrophies

77
Q

What is the least common stromal dystrophy?

A

Macular dystrophy

78
Q

What is the most severe stromal dystrophy?

A

Macular dystrophy

79
Q

What is macular dystrophy caused by?

A

Glycosaminoglycan deposits

80
Q

What is the mode f inheritance for macular dystrophy?

A

Autosomal recessive

81
Q

Which region is macular dystrophy more common?

A

Middle East

82
Q

What are the two subtypes of macular dystrophy and how is this achieved? Which is more common and what is the onset?

A

Typed through blood testing
Type I and II
Type I onset is in childhood and is more common

83
Q

Distinguish between type I and II macular dystrophy.

A

Type I lacks keratan sulphate in the cornea

Type II - keratan sulphates are present in the cornea

84
Q

How does macular dystrophy appear (region, colour, edge)? From where to where does it extend?

A

Central, grey-white, ill-defined but focal opacities

Extends from limbus to limbus

85
Q

Can macular dystrophy involve the entire stromal thickness?

A

Yesd eventually

86
Q

List 3 signs of macular dystrophy.

A

Reduced corneal thickness
Reduced corneal sensitivity
Corneal endothelium affected in late disease (guttatae)

87
Q

List a differential diagnosis for macular dystrophy and give three examples.

A

Other anterior stromal dystrophies
Reis-bucklers dystrophy
Granular dystrophy
Lattice dystrophy

88
Q

What is the treatment for macular dystrophy? Is recirrence common?

A

Referral for penetrating keratoplasty

Recurrence uncommon

89
Q

By when is vision significantly affected in macular dystrophy?

A

Third decade

90
Q

Is schnyders corneal dystrophy common or rare? What is the mode of inheritance and onset?

A

Rare
Autosomal dominant
Onset 2nd or 3rd decade

91
Q

What condition may schnyders corneal dystrophy be associated with?

A

Systemic hypercholesterolaemia

92
Q

In what form of schnyders corneal dystrophy is diagnosis more difficult?

A

Acrystalline form

93
Q

What is schnyders corneal dystrophy caused by? What defect in what cell and what this leads to (2)?

A

Caused by a metabolic defect in corneal keratocytes, leading to cholesterol deposition in the anterior stroma

94
Q

What does schnyders corneal dystrophy look like (colour, region, layer)?

A

White-yellow lipid deposits in the central anterior stroma

95
Q

What is schnyders corneal dystrophy often associated with (ocular and not systemic)?

A

Prominent corneal arcus

96
Q

List two symptoms of schnyders corneal dystrophy.

A

Increasing glare and reduced visiond in adulthood

97
Q

List two differential diagnoses for schnyders corneal dystrophy and give three examples for each.

A 
Other anterior stromal dystrophy
-reis-bucklers dystrophy
-granular dystrophy
-lattice dystrophy
Other causes of corneal crystals
-infectious crystalline keratopathy
-gout
-mutiple myeloma
98
Q

What is the treatment for schnyders corneal dystrophy (2)?

A

Refer for blood lipid workup

Referral for PTK in patients with severe glare

99
Q

Is a corneal graft typically required for schnyders corneal dystrophy?

A

Rarely

100
Q

What is the most common endothelial dystrophy?

A

Fuchs endothelial corneal dystrophy

101
Q

Which gender is more affected by fuchs endothelial corneal dystrophy?

A

Females>males

102
Q

Are cases withot inheritance common for fuchs endothelial corneal dystrophy? What is the mode of inheritance (2)?

A

Cases without inheritance most common
Autosomal dominant
Can be autosomal recessive

103
Q

When is fuchs endothelial corneal dystrophy typically symptomatic?

A

5th decade

104
Q

What is fuchs endothelial corneal dystrophy due to and what happens to endothelial cells as a result? What develops on descemets membrane and what is this called? What is the ultimate consequence of these events?

A

Due to diffuse thickening and lamination of descemets membrane
Endothelial cells become sparse and atrophic
Development of hyaline excrescences on the thickened membrane - corneal guttata
Progressive degeneration of corneal endothelial cells

105
Q

Is fuchs endothelial corneal dystrophy symptomatic early on?

A

Not typically

106
Q

List a symptom of moderate fuchs endothelial corneal dystrophy and why it occurs.

A

Mild decrease in VA which increases over time as the posterior stromal oedema increases

107
Q

List a symptom of late fuchs endothelial corneal dystrophy and why it occurs. When is it worse (time of the day)? Does it improve?

A

Significant reduction in VA due to epithelial oedema

Worse upon waking, which improves after several hours

108
Q

What may patients with late fuchs endothelial corneal dystrophy develop? What can this cause and why?

A

Epithelial bullae which can rupture, causing severe pain

109
Q

What two lesions can be seen with fuchs endothelial corneal dystrophy?

A

Epithelial microcysts and bullae

110
Q

List three differential diagnoses for fuchs endothelial corneal dystrophy.

A

Aphakic and pseudophakic bullous keratopathy (after cataract surgery)
Posterior polymorphous dystrophy

111
Q

When is a review for fuchs endothelial corneal dystrophy recommended?

A

6/12 months

112
Q

What are the three components to working up fuchs endothelial corneal dystrophy?

A

Pachymetry
Specular microscopy
IOP

113
Q

What are two treatment options for corneal oedema with fuchs endothelial corneal dystrophy?

A 
Hypertonic solution (5%)
Warm hairdryer over cornea upon waking
114
Q

What are two treatment options for bullae with fuchs endothelial corneal dystrophy?

A

Ocular lubricants

Bandage CLs

115
Q

What will eventually be required for fuchs endothelial corneal dystrophy (treatment)?

A

Referra for penetrating keratoplasty

116
Q

What two layers are affected in posterior polymorphous corneal dystrophy?

A

Descemets membrane and the endothelium

117
Q

What is the mode of inhertiance and onset for posterior polymorphous corneal dystrophy?

A

Autosomal dominant

Early childhood

118
Q

What appearance do corneal endothelial cells have with posterior polymorphous corneal dystrophy? What structure do they have and what do they stain positive for?

A

Appearance of epithelial cells

Have microvilli and stain for keratin

119
Q

What does posterior polymorphous corneal dystrophy increase the risk of and why?

A

Increased risk of glaucoma due to cells growing into the trabecular meshwork

120
Q

What condition may posterior polymorphous corneal dystrophy be associated with?

A

Alports syndrome

121
Q

How do the lesions of posterior polymorphous corneal dystrophy appear? Are they symmetric?

A

Grouped or linear bubble-like lesions within descemets membrane
Lesions are frequently asymmetric

122
Q

What may develop in advanced cases of posterior polymorphous corneal dystrophy?

A

Corneal oedema

123
Q

What can develop in ~25% of posterior polymorphous corneal dystrophy cases?

A

Peripheral irido-corneal adhesions

124
Q

Are symptoms of posterior polymorphous corneal dystrophy common? List 2 of them.

A

Uncommon

May develop reduced vision and ocular discomfort if corneal oedema develops

125
Q

List 2 differential diagnoses for posterior polymorphous corneal dystrophy. Explain differentiating factors for each if applicable.

A

Iridocorneal endothelial syndrome (unilateral, non-hereditary)
Fuchs endothelial dystrophy (late onset)

126
Q

What is the treatment for posterior polymorphous corneal dystrophy (3)?

A

Most dont require treatment for corneal changes
Must monitor IOP each visit
If treatment required, as per fuchs endothelial dystrophy

OD2 - Applied Clinical Training & Research Studies in Vision and Optometry (174 decks)

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