Block 15: Lectures 4-6 Flashcards

1
Q

In what age group does keratoconus typically present?

A

Teens and 20’s

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2
Q

Is keratoconus usually unilateral or bilateral?

A

Unilateral initially and becomes bilateral over time

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3
Q

What is the cause for blurred vision in keratoconus?

A

Irregular astigmatism

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4
Q

What is a rupture or tear in Descent’s Membrane that allows a sudden influx of aqueous into the cornea called?

A

Acute hydrops

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5
Q

What type of reflex is seen upon retinoscopy on a keratoconus patient?

A

Scissor reflex

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6
Q

What type of reflex is seen upon ophthalmoscopy on a keratoconus patient?

A

Oil droplet

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7
Q

Define “Vogt Striae” that are commonly seen when examining an individual with keratoconus?

A

Vertical stress lines deep within stroma that disappear when pressure is applied to globe

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8
Q

What is it called when the cornea takes a “cone-like” appearance that is observable from a side view or against the lower lid in downsize?
(Name the feature/sign, not the condition it may be associated with)

A

Munson sign

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9
Q

What location/direction does the protrusion or “cone” ultimately progress to on the cornea in keratoconus?

A

Infero-temporal

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10
Q

If present, where is Fletcher’s ring usually located on a keratoconus patient?

What filter on slit lamp allows best view of this?

A

Base of “cone”

Cobalt blue filter

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11
Q

What are 2 tests commonly used to confirm keratoconus?

A
  1. Keratometry

2. Corneal topography

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12
Q

What is the difference between an individual with keratoconus who has no complaints of plain and another keratoconus individual who has pain and photophobia?

A

Pain and photophobia signifies a ruptured Descemet’s Membrane (“Acute hydrops”)

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13
Q

Why might corneal cross-linking be used as a treatment strategy in keratoconus?

A

To stiffen collagen - stabilize and maybe even reverse the ectasia

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14
Q

What day to day motion involving the eye should an individual with keratoconus avoid?

A

Eye rubbing

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15
Q

What is a common keratometry reading on an eye with keratoconus?

A

50.00 D and above

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16
Q

What type of intracorneal implants might be used as a treatment/management option for keratoconus?

A

Ring segments

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17
Q

Which layer of the cornea becomes edematous with Acute Hydrops?

A

Stroma

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18
Q

How long does it typically take Acute Hydrops to spontaneously heal?

A

2-4 months

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19
Q

What can be used to draw fluid out of epithelium in Acute Hydrops?

A

Hypertonic saline drop

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20
Q

What are 3 options that could be used to manage pain and discomfort in acute hydrops?

A
  1. Cycloplegia
  2. Bandage contact lens
  3. NSAID
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21
Q

What surgical procedure may accelerate recovery and reduce scarring in Acute Hydrops?

A

Injection of anterior chamber (intracameral) with gas/air

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22
Q

Name the condition that involves peripheral corneal thinning from 4:00-8:00 without apical protrusion.

A

Pellucid marginal degeneration

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23
Q

What pattern is classically seen on a topography map in an individual with Pellucid Marginal Degeneration?

A

Butterfly or Kissing Birds

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24
Q

Although surgery is difficult in Pellucid Marginal Degeneration, what is currently the most favored technique?

A

Peripheral lamellar crescentic keratoplasty followed by central penetrating keratoplasty

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25
Q

What is a key differentiating feature between Keratoconus and Keratoglobus?

A

The shape of the corneal ectasia - cone in keratoconus and globular in keratoglobus

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26
Q

Where is the corneal thinning and protrusion typically located in Keratoglobus?

A

Diffuse

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27
Q

Name two conditions that intrastromal ring segments and corneal cross-linking have a helpful benefit.

Name one condition that they are not effective in treating/managing.

A

Beneficial:

  1. Keratoconus
  2. Pellucid Marginal Degeneration

Not Effective:
1. Keratoglobus

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28
Q

Which condition is most difficult to manage/treat?

Keratoconus, Pellucid Marginal Degeneration, or Keratoglobus?

A

Keratoglobus - spectacles and contact lenses often cannot correct vision and surgery is very difficult and often ineffective

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29
Q

What is a genetic eye disorder in which abnormal material often accumulates in the cornea and is usually progressive? (General term)

A

Corneal dystrophy

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30
Q

How are most forms of corneal dystrophies inherited?

A

Autosomal dominant

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31
Q

Are most corneal dystrophies bilateral or unilateral?

A

Bilateral

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32
Q

What is the international classification of a corneal dystrophy based on?

A

The affected chromosomal loci and it’s gene and mutation

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33
Q

What is the typical inheritance pattern in Epithelial Basement Membrane Dystrophy?

A

Sporadic (much more commonly than Autosomal dominant)

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34
Q

Where is the accumulation of material in Epithelial Basement Membrane Dystrophy?

A

Between basement membrane and Bowman’s layer

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35
Q

“Maps, dots, and fingerprints” are often used to describe signs seen in what condition?

A

Epithelial Basement Membrane Dystrophy

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36
Q

Where are the intraepithelial cysts most prominent in Meesmann’s Epithelial Dystrophy?

A

Interpalpebral zone

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37
Q

What is bowman’s layer replaced with in Reis-Bucklers’ Corneal Dystrophy?

A

Connective tissue bands

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38
Q

What feature of Reis-Bucklers’ Corneal Dystrophy typically spontaneously presents during early childhood?

A

Recurrent corneal erosions

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39
Q

What is considered a less severe version of Reis-Bucklers’ Corneal Dystrophy?

A

Thiel-Behnke corneal dystrophy

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40
Q

What treatment option should usually be tried before keratoplasty in Reis-Bucklers’ Corneal Dystrophy?

A

Laser keratectomy

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41
Q

What makes keratoplasty less ideal and saved for last option in Reis-Bucklers’ Corneal Dystrophy?

A

Condition can recur on the graft

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42
Q

How do the connective tissue bands appear in Reis-Bucklers’ Corneal Dystrophy?

A

Coarse grey/white subepithelial deposits

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43
Q

What layer of the cornea are deposits found in Lattice Corneal Dystrophy?

A

Stroma

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44
Q

What abnormal material accumulates in Lattice Corneal Dystrophy?

A

Amyloid

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45
Q

What could be the cause of progressively impaired vision in Lattice Corneal Dystrophy?

A

Filamentous appearance of amyloid deposits across cornea

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46
Q

How is opacification usually treated in Lattice Corneal Dystrophy?

A

Penetrating or lamellar keratoplasty

47
Q

What condition is characterized by white deposits resembling sugar granules that gradually coalesce?

A

Granular Corneal Dystrophy

48
Q

What substance is accumulated in Granular Corneal Dystrophy?

In what layer of the cornea?

A

Hyaline

Stroma

49
Q

What are the two types of Granular Corneal Dystrophy referred to as?

A

Type 1: Classic

Type 2: Avellino

50
Q

What feature does Type 2 (Avellino) Granular Corneal Dystrophy have that Type 1 does not?

A

Hyaline + Amyloid deposits

Type 1 has Hyaline only

51
Q

What is an example of an autosomal recessive corneal dystrophy that is relatively common in Iceland?

A

Macular Corneal Dystrophy

52
Q

What material is accumulated in Macular Corneal Dystrophy?

A

Glycosaminoglycans

53
Q

How is Macular Corneal Dystrophy usually treated?

A

Penetrating keratoplasty

54
Q

A disorder of _______ metabolism is responsible for the deposits found in Schnyder Corneal Dystrophy?

A

Lipid

55
Q

What material accumulates in Schnyder Corneal Dystrophy?

A

Phospholipids and cholesterol

56
Q

What treatment is used for Schnyder Corneal Dystrophy?

A

Excimer/laser keratectomy or keratoplasty

57
Q

Accelerated endothelial loss is called ______?

A

Fuchs’ Endothelial Dystrophy

58
Q

What is the typical inheritance pattern for Fuchs’ Endothelial Dystrophy?

A

Sporadic

59
Q

When is the blurred vision typically worse in Fuchs’ Endothelial Dystrophy?

A

Morning

60
Q

Specular reflection typically shows the appearance of what sign in earlier phases of Fuchs’ Endothelial Dystrophy?

A

Guttata

61
Q

In Fuchs’ Endothelial Dystrophy, “guttata” often progress to what clinical sign?

A

“Beaten metal” appearance of endothelium

62
Q

What effect is seen first in Fuchs’ Endothelial Dystrophy, stromal or epithelial edema?

A

Stromal edema, causing blur

63
Q

What clinical sign is apparent when Fuchs’ Endothelial Dystrophy has progressed from stromal blur to epithelial?

A

Microcysts/bullae

64
Q

What would be the cause of acute pain in later stages of Fuchs’ Endothelial Dystrophy?

A

Rupturing microcysts/bullae causing exposed nerve endings

65
Q

Why might a hairdryer be used to manage Fuchs’ Endothelial Dystrophy?

A

To dehydrate cornea

66
Q

What two surgical procedures have a high success rate in Fuchs’ Endothelial Dystrophy?

A
  1. Posterior lamellar keratoplasty

2. Posterior penetrating keratoplasty

67
Q

How would cataract surgery affect Fuchs’ Endothelial Dystrophy?

A

Cataract surgery would make it worse due to loss of more endothelial cells

68
Q

What is included in a “triple procedure” for a patient with Fuchs’ Endothelial Dystrophy?

A
  • Cataract surgery
  • Lens implantation
  • Keratoplasty
69
Q

What are 3 ways to dehydrate cornea in Fuchs’ Endothelial Dystrophy?

A
  1. 5% NaCl drops/ointment
  2. Decrease IOP
  3. Hairdryer
70
Q

What cells are abnormally developed in Posterior Polymorphous Corneal Dystrophy?

A

Endothelial cells

71
Q

Abnormal endothelial cells have the characteristics of what type of cell in Posterior Polymorphous Corneal Dystrophy?

A

Epithelial

72
Q

What two conditions should be watched for in Posterior Polymorphous Corneal Dystrophy?

A
  1. Peripheral anterior synechiae

2. Glaucoma

73
Q

What are 3 appearances the endothelial lesions may have in Posterior Polymorphous Corneal Dystrophy?

A
  1. Vesicular
  2. Band-like
  3. Diffuse
74
Q

If opacification occurs in Posterior Polymorphous Corneal Dystrophy, what treatment is preferred?

A

Deep lamellar keratoplasty

75
Q

What is an involutional (age-related) change to the eye that tends to affect the peripheral portion of the cornea more than the central? (General term)

A

Corneal Degeneration

76
Q

Are corneal degenerations more often unilateral or bilateral?

A

Unilateral (asymmetric when bilateral)

77
Q

Lipid deposits in a circumferential stromal band seen in a young patient with dyslipidemia is known as ______? (specific!)

A

Corneal Arcus - Arcus Juvenilis

78
Q

Lipid deposits in peripheral stroma in an elderly patient without any predisposing systemic condition is known as ______? (specific!)

A

Corneal Arcus - Arcus Senilis/Gerontoxon

79
Q

What is a key difference between corneal lipid deposits in Schnyder Corneal Dystrophy and Corneal Arcus (Sinilis)?

A

Location of deposits

  • Central in Schnyder corneal dystrophy
  • Peripheral in Arcus Sinilis
80
Q

If an elderly patient has unilateral Corneal Arcus with no other known conditions, what should be checked for?

A

Carotid disease on the uninvolved side

81
Q

What is the condition that presents in the majority of those over 40 years old as peripheral interpalpebral whitish crescents?

A

Vogt Limbal Girdle

82
Q

Vogt Limbal Girdle has histological changes similar to _____?

A
  1. Pinguecula

2. Pterygium

83
Q

Where are the deposits found in Corneal farinata?

A

Deep stroma, most prominent centrally

84
Q

What illumination technique is best for observing Corneal Farinata?

A

Retroillumination

85
Q

What layer of the cornea is most frequently involved in Crocodile shagreen?

A

Anterior 2/3 stroma

86
Q

What condition is characterized by grayish-white polygonal stromal opacities separated by relatively clear spaces?

A

Crocodile shagreen

87
Q

What condition is defined by deposits of calcium within and adjacent to Bowman’s layer?

A

Band Keratopathy

88
Q

What material is deposited in the cornea in Band Keratopathy?

Which layer of the cornea?

A

Calcium

Bowman’s Layer

89
Q

Where does the plaque of calcium typically begin forming in Band Keratopathy? (not referring to layer of cornea)

A

Nasal and temporal, extending centrally

90
Q

What are two ways Band Keratopathy could be ocular-caused?

A
  1. Chronic inflammation/edema

2. Silicone oil in Anterior chamber

91
Q

When is treatment indicated in Band Keratopathy?

A

When decreased vision and pain are present, due to epithelial breakdown

92
Q

If treatment is needed for Band Keratopathy and the treating doctor uses EDTA during the procedure, what procedure was most likely performed?

A

Chelation

93
Q

Other than calcium, what else could be elevated in serum levels if a metabolic defect is the cause of Band Keratopathy?

A

Phosphate

94
Q

What forms nodules in Salzmann’s Nodular Degeneration?

A

Hyaline

95
Q

A rare autosomal recessive disorder that results in the accumulation of cystine crystals in many organs throughout the body is known as _____?

A

Cystinosis

96
Q

What are the three forms of Cystinosis?

A
  1. Infantile
  2. Late-onset juvenile
  3. Adult
97
Q

Which form of Cystinosis typically does not involve renal failure?

A

Adult

98
Q

What part of the cornea does Cystinosis start and then progress to?

A

Starts in anterior periphery and extends posteriorly and centrally as it progresses

99
Q

How might earlier onset forms of Cystinosis affect ocular tissues in ways that late-onset might now?

A

Pigmentary retinopathy

100
Q

What treatment is given as soon as possible after diagnosis of infantile or juvenile cystinosis?

A

Oral cysteamine

101
Q

A rare autosomal recessive condition involving abnormal deposition of copper in the liver, brain and cornea is known as ____?

A

Wilson Disease

102
Q

What is the most common ocular manifestation of Wilson Disease?

A

Kayser-Fleischer ring

103
Q

What are two systemic manifestations commonly seen in Wilson Disease?

A
  1. Liver dysfunction

2. Parkinsonism

104
Q

What layer does copper accumulate in with Wilson Disease?

A

Descent’s membrane - around corneal limbus

105
Q

Are Kayser-Fleischer rings more commonly present when there is neurologic involvement or liver involvement?

A

Neurologic (Greater than 90%)

106
Q

What clinical finding in the lens is also associated with Wilson disease?

A

Sunflower cataract

107
Q

What are three systemic treatments for Wilson Disease?

A
  1. Penicillamine
  2. Zinc
  3. Ammonium tetrathiomolybdate
108
Q

What is an X-linked recessive disorder in which an enzyme deficiency leads to tissue accumulation of a glycolipid?

A

Fabry disease

109
Q

What is an early ocular sign that most patients develop in Fabry Disease?

A

Vortex keratopathy (Cornea Verticillata or Whorl Keratopathy)

110
Q

Why is the diagnosis Fabry Disease often overlooked until later in life?

A

Early signs/symptoms often dismissed as “growing pains”

Episodic pain in hands/feet during childhood

111
Q

If a diagnosis of Fabry Disease has been missed (when the individual does in fact have it), what may lead to a diagnosis when the patient reaches 30-40?

A

Kidney or heart problems

112
Q

What medications can be used to treat Fabry disease?

A

Pain meds and ERT

113
Q

A wedge or spoke-shaped posterior cataract is known as ______?

A

Fabry cataract