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Diseases of Anterior Seg. > Cornea part 2: corneal dystrophies > Flashcards

Cornea part 2: corneal dystrophies Flashcards

1
Q

define dystrophy (traditional)

A

inherited corneal disease, usually one layer of cornea is involved, tend to affect central cornea

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2
Q

T/f IC3D intergrated an up to date info in phentotypic description, pathologic examination and genetic anaylsis .

A

true . Grouped in categories based on genetic evidence supporting the existence of a dystrophy

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3
Q

Epithelial Basement membrane dystrophy is in what layer of cornea?

A

epithelial and subepithelia dystrophies

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4
Q

Messmann Corneal dystrophy is in what layer of cornea?

A

epithelial and subepithelia dystrophies

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5
Q

Reis Buckler Corneal dystrophy is in what layer of cornea?

A

bowman layer

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6
Q

Theil-behnke Corneal Dystrophy is in what layer of cornea?

A

bowman layer

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7
Q

lattice corneal dystrophy is in what layer of cornea?

A

stroma

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8
Q

granular corneal dystrophy is in what layer of cornea?

A

stroma

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9
Q

macular corneal dystrophy is in what layer of cornea?

A

stroma

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10
Q

schnyder corneal dystrophy is in what layer of cornea?

A

stroma

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11
Q

fleck corneal dystrophy is in what layer of cornea?

A

stroma

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12
Q

Fuch’s endothelial corneal dystrophy is in what layer of cornea?

A

descemet membrane and endothelial dystrophies

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13
Q

another name for epithelial basement membrane dystrophy (ebmd) is

A

mapped dot fingerprint dystrophy or anterior basement membrane dystrophy or cogans microcystic dystrophy

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14
Q

most cases of epithelial basement membrane dystrophy have MANY inheritances documented

A

false. EBMD has NO inheritance documented.

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15
Q

Which corneal dystrophy shows up later in life in adulthood?

A

ebmd

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16
Q

is ebmd is many considered _________

A

degenerative. The location and defree of pathology can flunctuate over time too.

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17
Q

which dystrophy is rarely seen in children?

A

ebmd

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18
Q

Abnormality of production of epithelial basement membrane that extends into epithelium is _________

A

ebmd. Multiple basement membranes in the epithelial layer

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19
Q

MOST common corneal dystrophy

A

EBMD

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20
Q

a BIG clue for EBMD is _____ during slit lamp examination

A

NEGATIVE staining

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21
Q

t/f . Patients with EBMD are asymptonmatic and we just watch them over time.

A

true.

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22
Q

T/F you do need all three (maps, dots, fingerprints) for it to be EBMD .

A

false. You just need at least one of them.

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23
Q

A consequence of EBMD IS _____

A

RCE. Patients can experience pain, tearing, blurred vision

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24
Q

Management for EBMD includes

A

freshKote, muro 128 (stings) {hypertonic saline drops/ointments}, bandage CL, amniotic membrane, lubricants

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25
Q

Which is more appropiate for EBMD lamellar kertatoplasty or penetrating keratoplasty?

A

lamellar Kertaplasty. Only small portion of cornea involved.

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26
Q

a DDX for EBMD is low TBUT. How do you tell a low tbut from negative staining?

A

negative staining will occur in the same place everytime. Low tbut will vary

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27
Q

Messman Corneal dystrophy clinical description is

A

100s tiny vesicles (cysts) develop in epithelium . These vesicles extend to limbus and most numerous in interpalpebral zone

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28
Q

which corneal dystrophy may be asymptomatic ?

A

both epithelial (messamans, and EBMD), PPMD

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29
Q

how to differenitate messman’s vs ebmd vs cysts from CL wear?

A

mesamans are uniform in size, bilateral, and diffuse distribution. These patients also have light/glare sensitivity

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30
Q

What’s the management for messaman?

A

lubricants, manage RCE

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31
Q

When does RCE develop in Reis Bucklers dystrophy?

A

in first 1-2 years of life. Reason why its blurry vision from childhood

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32
Q

Reis Buckler affects which area of the bowman layer?

A

central and midperipheral cornea

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33
Q

fine reticular pattern opacities in bowmans layer and superficial stroma is

A

reis bucklers . Opacities start separated and become confluent over time

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34
Q

which of the following are appropiate to treat reis bucklner? A. superficial keratectomy b. ptk c. lamellar or pentrating keratoplasty. D. all of above

A

d. all of above. Need the superificial keratectomy

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35
Q

easy way to remember reis appearance

A

pirates have netlike cargo. And “b” stands for bowman

36
Q

how can you differiante thiel behnke from reis buckner? HINT: rce onset.

A

rce in thiel-behnke develops IN 1ST- 2ND DECADE of life. Reis buckner develops in 1st to 2nd YEARS of life

37
Q

another name for thiel behnke is

A

honeycomb shpaed corneal dystrophy

38
Q

Which area of the cornea is typically uninvolved in thiel behnke?

A

periheral. They are symmetrical reicular opacities that can progress to deep stromal layers and corneal periphery/

39
Q

easy way to remember lattice dystrophy.

A

lettuce. Looks like the veins on the lettuce. In stroma.

40
Q

this dystrophy has gray branching lines in stroma, discrete subepi white dots AND central anterior stomal haze

A

lattice dystrophy type I

41
Q

MOST COMMON corneal STOMAL dystrophy

A

lattice dystrophy type I

42
Q

lattice usually appears by end of _____decade

A

1st decade of life.

43
Q

the lines in lattice dystrophy type 1 start centrally. True/false

A

true. Start central and more superifically and then spreads periph. And go deeper

44
Q

which dystrophy needs an EARLY kertoplasty (lamelly or penetrating)?

A

lattice dystrophy type I. they need it by 4th decade of life. They have frequent RCE

45
Q

lattice dystophy type II is truly ____ and not a dystrophy.

A

systemic disorder, familial amyloid polyneuropathy.

46
Q

Lattice type II dystrophy onset’s is _____ and they have an increased risk of ____

A

3rd - 4th decade of life. Increased risk of POAG.

47
Q

unique about the visual acuity in lattice type II ?

A

their VA’s are normal until 6th decade of life. - because their central cornea is relatively SPARED. Unlike type I. gray lines are more in periphery.

48
Q

another name for granular corneal dystrophy type I

A

Groenouw’s Corneal Dystrophy Type 1

49
Q

easy way to remember granualar corneal dystrophy appearance

A

bread crumbs .

50
Q

t/f the periphery is involved in granular corneal dystrophy.

A

f. its not involved at all.

51
Q

______ has onset in childhood – may be seen as early as 2 years of age

A

Groenouw’s Corneal Dystrophy Type 1

52
Q

managemet for granular type I

A

manage rce, ptk, lamellar or penetrating keratoplasty

53
Q

granular corneal dystrophy type II is also known as

A

Avellino Dystrophy

54
Q

significant amyloid deposition is in _____ dystrophy.

A

granular corneal dystrophy type II

55
Q

most sever of STOMAL dystrophy

A

macular corneal dystrophy

56
Q

Groenouw’s Corneal Dystrophy Type 2 is

A

macular corneal dystrophy

57
Q

poorly demarcated gray-white opacities with diffuse cloudiness in intervening stroma

A

macular corneal dystrophy

58
Q

macular corneal dystrophy tends to run in

A

families. Common in descendants from Iceland. Autosomal recessive

59
Q

avellino dystrophy can involve all layers of the cornea.

A

false. Macular corneal dystrophy can involve alll layers of cornea.

60
Q

management for macular corneal dystrophy

A

penetrating keratoplasty (good prognosis ) rce management

61
Q

Schnyder’s Corneal Dystrophy is aka

A

Schnyder Crystalline Corneal Dystrophy

62
Q

central corneal haze and/or fine, ring of yellowish-white crystalline cholesterol deposits in central anterior stroma

A

Schnyder Crystalline Corneal Dystrophy

63
Q

Name three conditions that involve hyperlipidemia

A
  1. arcus senilis 2. schynders corneal dystrophy 3.xanthoma
64
Q

an easy way to remmeber if schynders has crystals everytime.

A

schnyders prezetals can come with salt or without salt.

65
Q

management of schynders

A

lipid panel.

66
Q

Which two corneal dystrophys DO NOT have RCE as a symptom ?

A

Schnyder Crystalline Corneal Dystrophy and posterior polymorphus corneal dystrophy (PPMD)

67
Q

corneal gutta is

A

Degenerating endothelial cells produce localized nodular thickenings of Descemet’s membrane (endothelial excrescences) – these are called guttata

68
Q

Centrally guttata are associated with Endothelial and Fuchs’ Dystrophies

69
Q

there is no edema in fuch’s dystrophy

A

false. Theres no edema in endothelial dystrophy.

70
Q

endotheilial and fuchs dystrophy both have

A

corneal guttata (endothelial excrescences), and pigment on endothelium

71
Q

no txt is needed in _____ (fuchs or endothelial)

A

endothelial

72
Q

fuchs is more commen in ___ (men or women)

73
Q

FUCHS involves stomal edma, epithelial edema, and sometimes subepiethelial fibrosis

74
Q

Endothelial celss undergo _____ (change in cell size) AND _____ (change in shape).

A

polymegathism and pleomorphism

75
Q

A contraindication for fuchs is _______ surgery

76
Q

PT complains of blurry vision in am and clears away throughout day, they are female, and have bullae keratopathy (cysts coalse to form bullae -blisters in epitheilium)

A

fuchs dystrophy.

77
Q

WHAT plan is going to be for fuchs

A

fresh kote, glaucoma med to lower IOP, endothelial keratoplasy or penetrating)

78
Q

Band-like structures (“railroad tracks”) is what type of dystrophy

A

Posterior Polymorphous Corneal Dystrophy

79
Q

Nodular grouped vesicular and blister-like lesions on endothelial surface

A

Posterior Polymorphous Corneal Dystrophy

80
Q

Posterior Polymorphous Corneal Dystrophy is at risk for _____

A

glaucoma. Because abnormal cells may extend into trabecular meshwork

81
Q

patients with PPMD are symtomatic and require penetrating/endothelial keratoplasty.

A

false. Ppcd pts are ASYMPTOMATIC and rarely require surgery. Management inovles glaucoma meds if they have glaucoma.

82
Q

what is desek ? And which dystrophy is it appropiate for>

A

descemets stripping endothelial kertoplasty. Lamellar kertoplast of the ENDOTHELIUM.

83
Q

t/f. PPMD is not assoicated with guttata but does cause RCE.

A

flase. PPMD is not associated with gutta and does cause RCE.

84
Q
A

dots are irregular round, oval or comma shaped non staining putty gray opacities in epithelium

85
Q
A

maps irregular islands of thickened, gray hazy epithelium scalloped, circumscribed borders.

86
Q
A

fingerprints are parallel,curvilinear lines, usually paracentral in the epithelium

Diseases of Anterior Seg. (11 decks)

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