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Cornea & Lens and Cataract > Dystrophies > Flashcards

Dystrophies Flashcards

1
Q

What are the best slit lamp examining techqunies to see MDF?

A
  1. Sclerotic scatter 2. retroillumination 3. Broad tangential beam
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2
Q

What happens to the cornea in Meesmanns?

A

Slightly thinned and sensation may be reduced.

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3
Q

Which dystrophy is X linked with gray, band shaped feathery lesions in whorled patterns with densely crowded clear microcysts?

A

Lisch (pain free)

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4
Q

Which dystrophy has painful recurrent epithelial erosions with geographic or honeycomb gray white reticular opacification in the central cornea?

A

Reis-Bucklers (sawtooth Bowman’s layer)

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5
Q

How do you diferentie Reis-Bucklers from Thiel-Behnke dystrophy?

A

RB - rod-shaped bodies; TB - has curly fibers

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6
Q

Which is the most common stromal dystrophy?

A

Granular

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7
Q

What are the 4 stains for Amyloid (Lattice)?

A
  1. Congo red
  2. Crystal violet
  3. Thioflavine T
  4. Masson trichrome stain
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8
Q

What dystrophy stain bright red with rod-shaped bodies immersed in an amorphous matrix?

A

Granular

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9
Q

Which dystrophy involves the corneal periphery and endothelium?

A

Macular

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10
Q

What are the 2 recessive corneal dystrophies?

A
  1. Maclar 2. Gelatinous droplike
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11
Q

Which stromal dystrophy reoccurs in PK grafts?

A

Lattice (also gelatinous droplike and reis bucklers)

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12
Q

Schnyder crystalline corneal dystrophy stains with what and has what systemic d/o?

A

Oil red O stains the phospholipids red; Hyperlipopreteima and hperlipidemia; decreased corneal sesnsation.

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13
Q

Which dystrophy has a extreme asymmetric presentation with decreased corneal sensation, limbal mermaids, keratoconus, punctate cortical lens changes, pseudoxanthoma elasticum, and atopy?

A

FLECK

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14
Q

What are the genetics of Pellucid Marginal Degeneration?

A

Uncommon, nonherediatry, bilateral

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15
Q

Where is the thinning greatest in Keratoglobus?

A

In the PERIPHERY

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16
Q

Which 2 lysosomal storage diseases (mucopolysaccharidosis) have mainly corneal involvement?

A
  1. Sheie 2. Hurlers
17
Q

Which dystrophy has peripheral, sparkling, yellow-white spots at the corneal limbus in the superficial stroma that may fade with time?

A

Bieetti crystalline corneoretinal dystrophy

18
Q

Which dystrophy has photophobia with crystals in the corneal stroma and TM and can cause death in the infantile form but adults form life expectancy is normal?

A

Cystinosis

19
Q

List 6 causes of ENLARGED corneal nerves

A
  1. MEN IIB
  2. Phytanic acid storage disease (Refsum syndrome)
  3. Hansen disease
  4. Riley-Day syndrome
  5. NF
  6. Acanthamoeba perineuritis
20
Q

List 5 causes for MORE VISIBLE corneal nerves?

A
  1. Keratoconus
  2. Ichthyosis
  3. Fuchs corneal dystrophy
  4. Corneal edema
    5 Congential GL
21
Q

What are the 11 causes of corneal crystals?

A
  1. Macular dystrophy
  2. Granular dystrophy
  3. Lattice dystrophy
  4. Schynder’s dystrophy
  5. Beitti’s dystrophy
  6. Ciloxin
  7. Strep viridans
  8. Dieffanbachia plant
  9. cystinosis
  10. Multiple myeloma
  11. MONOCLONAL GAMAOPATHY
22
Q

Fleck dystrophy is inherited?

A

AD - highly asymmetric and possibly U/L

23
Q

Which stromal dystrophy presents the earliest?

A

Macular - followed by lattice then granular.

24
Q

Lisch dystrophy affects what part of the cornea, what inheritance, and what makes it distinctive?

A

X-linked DOMINANT; epithelial; densely crowded epithelial microcysts-band shaped in whorled feathery pattern

25
Q

Abnl levels of keratin sulfate and dermatin sulfate is seen in which dystrophy?

A

Macular (type 1-no keratin; type 2 - decreased dermatin)

26
Q

Mutated cytokeratin is seen in what dystrophy?

A

Meesman’s (peculiar substance)

27
Q

Mutated kerato-epithelin is seen in what dystrophy?

A

BIGH3 - chr 5

28
Q

What stromal dystrophy extends anteriorly into the epithelium but not posteriorly?

A

Granular

29
Q

Fleck dystrophy is contained of what and stains with what?

A

MPS - Alcian blue and colloidal iron

Lipids - sudan black and oil red O

30
Q

Fleck Dystrophy is a/w what 7 d/o?

A
  1. Decreased corneal sensation
  2. Limbal dermoids
  3. KCN
  4. Central cloudy dystrophy
  5. Punctate cortical lens changes
  6. PXE
  7. ATOPY
31
Q

Reoccurrence in PKP incidence order?

A

Reis-bucklers > lattice > macular > granular

32
Q

What has 1/3

Of pts a/w hyperlipidemia or hyperchol?

A

Schynder’s central corneal dysrtophy

Cornea & Lens and Cataract (11 decks)

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