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

Cornea part 2: corneal dystrophies Flashcards

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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
Which is more appropiate for EBMD lamellar kertatoplasty or penetrating keratoplasty?
lamellar Kertaplasty. Only small portion of cornea involved.
26
a DDX for EBMD is low TBUT. How do you tell a low tbut from negative staining?
negative staining will occur in the same place everytime. Low tbut will vary
27
Messman Corneal dystrophy clinical description is
100s tiny vesicles (cysts) develop in epithelium . These vesicles extend to limbus and most numerous in interpalpebral zone
28
which corneal dystrophy may be asymptomatic ?
both epithelial (messamans, and EBMD), PPMD
29
how to differenitate messman's vs ebmd vs cysts from CL wear?
mesamans are uniform in size, bilateral, and diffuse distribution. These patients also have light/glare sensitivity
30
What's the management for messaman?
lubricants, manage RCE
31
When does RCE develop in Reis Bucklers dystrophy?
in first 1-2 years of life. Reason why its blurry vision from childhood
32
Reis Buckler affects which area of the bowman layer?
central and midperipheral cornea
33
fine reticular pattern opacities in bowmans layer and superficial stroma is
reis bucklers . Opacities start separated and become confluent over time
34
which of the following are appropiate to treat reis bucklner? A. superficial keratectomy b. ptk c. lamellar or pentrating keratoplasty. D. all of above
d. all of above. Need the superificial keratectomy
35
easy way to remember reis appearance
pirates have netlike cargo. And "b" stands for bowman
36
how can you differiante thiel behnke from reis buckner? HINT: rce onset.
rce in thiel-behnke develops IN 1ST- 2ND DECADE of life. Reis buckner develops in 1st to 2nd YEARS of life
37
another name for thiel behnke is
honeycomb shpaed corneal dystrophy
38
Which area of the cornea is typically uninvolved in thiel behnke?
periheral. They are symmetrical reicular opacities that can progress to deep stromal layers and corneal periphery/
39
easy way to remember lattice dystrophy.
lettuce. Looks like the veins on the lettuce. In stroma.
40
this dystrophy has gray branching lines in stroma, discrete subepi white dots AND central anterior stomal haze
lattice dystrophy type I
41
MOST COMMON corneal STOMAL dystrophy
lattice dystrophy type I
42
lattice usually appears by end of \_\_\_\_\_decade
1st decade of life.
43
the lines in lattice dystrophy type 1 start centrally. True/false
true. Start central and more superifically and then spreads periph. And go deeper
44
which dystrophy needs an EARLY kertoplasty (lamelly or penetrating)?
lattice dystrophy type I. they need it by 4th decade of life. They have frequent RCE
45
lattice dystophy type II is truly ____ and not a dystrophy.
systemic disorder, familial amyloid polyneuropathy.
46
Lattice type II dystrophy onset's is _____ and they have an increased risk of \_\_\_\_
3rd - 4th decade of life. Increased risk of POAG.
47
unique about the visual acuity in lattice type II ?
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
another name for granular corneal dystrophy type I
Groenouw’s Corneal Dystrophy Type 1
49
easy way to remember granualar corneal dystrophy appearance
bread crumbs .
50
t/f the periphery is involved in granular corneal dystrophy.
f. its not involved at all.
51
\_\_\_\_\_\_ has onset in childhood – may be seen as early as 2 years of age
Groenouw’s Corneal Dystrophy Type 1
52
managemet for granular type I
manage rce, ptk, lamellar or penetrating keratoplasty
53
granular corneal dystrophy type II is also known as
Avellino Dystrophy
54
significant amyloid deposition is in _____ dystrophy.
granular corneal dystrophy type II
55
most sever of STOMAL dystrophy
macular corneal dystrophy
56
Groenouw’s Corneal Dystrophy Type 2 is
macular corneal dystrophy
57
poorly demarcated gray-white opacities with diffuse cloudiness in intervening stroma
macular corneal dystrophy
58
macular corneal dystrophy tends to run in
families. Common in descendants from Iceland. Autosomal recessive
59
avellino dystrophy can involve all layers of the cornea.
false. Macular corneal dystrophy can involve alll layers of cornea.
60
management for macular corneal dystrophy
penetrating keratoplasty (good prognosis ) rce management
61
Schnyder’s Corneal Dystrophy is aka
Schnyder Crystalline Corneal Dystrophy
62
central corneal haze and/or fine, ring of yellowish-white crystalline cholesterol deposits in central anterior stroma
Schnyder Crystalline Corneal Dystrophy
63
Name three conditions that involve hyperlipidemia
1. arcus senilis 2. schynders corneal dystrophy 3.xanthoma
64
an easy way to remmeber if schynders has crystals everytime.
schnyders prezetals can come with salt or without salt.
65
management of schynders
lipid panel.
66
Which two corneal dystrophys DO NOT have RCE as a symptom ?
Schnyder Crystalline Corneal Dystrophy and posterior polymorphus corneal dystrophy (PPMD)
67
corneal gutta is
Degenerating endothelial cells produce localized nodular thickenings of Descemet’s membrane (endothelial excrescences) – these are called guttata
68
Centrally guttata are associated with Endothelial and Fuchs’ Dystrophies
TRUE
69
there is no edema in fuch's dystrophy
false. Theres no edema in endothelial dystrophy.
70
endotheilial and fuchs dystrophy both have
corneal guttata (endothelial excrescences), and pigment on endothelium
71
no txt is needed in _____ (fuchs or endothelial)
endothelial
72
fuchs is more commen in ___ (men or women)
women
73
FUCHS involves stomal edma, epithelial edema, and sometimes subepiethelial fibrosis
true.
74
Endothelial celss undergo _____ (change in cell size) AND _____ (change in shape).
polymegathism and pleomorphism
75
A contraindication for fuchs is _______ surgery
cataract
76
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)
fuchs dystrophy.
77
WHAT plan is going to be for fuchs
fresh kote, glaucoma med to lower IOP, endothelial keratoplasy or penetrating)
78
Band-like structures (“railroad tracks”) is what type of dystrophy
Posterior Polymorphous Corneal Dystrophy
79
Nodular grouped vesicular and blister-like lesions on endothelial surface
Posterior Polymorphous Corneal Dystrophy
80
Posterior Polymorphous Corneal Dystrophy is at risk for \_\_\_\_\_
glaucoma. Because abnormal cells may extend into trabecular meshwork
81
patients with PPMD are symtomatic and require penetrating/endothelial keratoplasty.
false. Ppcd pts are ASYMPTOMATIC and rarely require surgery. Management inovles glaucoma meds if they have glaucoma.
82
what is desek ? And which dystrophy is it appropiate for\>
descemets stripping endothelial kertoplasty. Lamellar kertoplast of the ENDOTHELIUM.
83
t/f. PPMD is not assoicated with guttata but does cause RCE.
flase. PPMD is not associated with gutta and does cause RCE.
84
dots are irregular round, oval or comma shaped non staining putty gray opacities in epithelium
85
maps irregular islands of thickened, gray hazy epithelium scalloped, circumscribed borders.
86
fingerprints are parallel,curvilinear lines, usually paracentral in the epithelium

Diseases of Anterior Seg. (11 decks)

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