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Flashcards in Cornea and External Dz Deck (251):
1

what is the mainstay treatment of pinguecula?

Lubrication with artificial tears and ointment can help with ocular surface irritation.

2

What are indications to remove pinguecula?

cosmetically unacceptable, chronic inflammation (can try short course of steroid drops), and if interferes with wearing contact lenses

3

pterygium. what's the most common age, gender, population, position on eye (why?)

most common in 20-30 men, along the equator, mostly nasally as it's theorized that the shadow of the nose prevents corneal refraction to temporal side.

4

what are concretions?

conjunctival epithelial inclusion cysts that are filled with keratin debris and mucopolysaccharide and mucin.

5

what is stocker line?

a pigmented line of iron deposition at the invasive border of a pterygium.

6

Indications for pterygium removal

cosmetic
inducing astigmatism, invasion of visual axis/blurry vision
constricting eye movements
Chronic inflammation--can try short course of steroids but not long term

7

what are the three muscles and respective innervations for eye lids?

orbicularis oculi: innervated by CNVII
Levator palpebrae: CN III
Muller muscles by symmathetic nerves

8

what is avg blinking rate?

10-15

9

What kind of glands are Glands of Moll

apocrine sweat glands

10

What kind of glands are Glands of Zeis

modified sebaceous glands

11

what are the afferent and efferent components of the lacrimal functional unit (LFU)?

CN V nociceptors on the ocular surface. to brain stem then efferent to meibomian gland, lacrimal gland, and goblet cells.

12

what does the caruncle attach to?

plica semilunaris

13

what is the blood supply of bulbar conj?

anterior ciliary arteries of the ophthalmic artery

14

conj associated Lymphoid tissue (CALT) is located where?

substantia propria of the conj

15

cornea contributes to roughtly how many % of the total refractive power?

74%

16

how thick is the corneal epithelial layer? what are the three components?

40-50 microns thick. superficial squamous, broad wing cells, columnar basal cells (1 layer)

17

Basal epithelial cells secrete a basement membrane of 50 nm thick. what kind of collagen is it?

Type IV

18

what is the make up of bowman's layer?

it's an acellular condensate to the anterior stroma

19

How thick if bowman's layer?

15 microns

20

What's the purpose of bowman's layer?

help to maintain shape of cornea

21

what are the major collagen and proteoglycan components of corneal stroma?

Collagen: type I, V fibrillary collagen interwoven with Type IV
Proteoglycans: decorin and lumican

22

What % is water in corneal stroma?

78%

23

what are the components of corneal hydration?

Na-K ATPase, swelling pressure of cornea due to glycosaminoglycans, and IOP compression, epithelial evaporation

24

where is the Dua's layer of cornea?

predecemet's layer

25

what structure is the "end" or edge of decement's membrane

schwalbe line

26

vertical and horizontal diameter of cornea in adults?

vertical 10-11; horizontal 11-12

27

which nerve innervates the cornea?

long ciliary nerve forms sub epithelial plexus

28

what technique can be used to visualize endothelial cells

specular microscopy

29

do endothelial cells proliferate?

not in vivo. damaged areas are covered by enlargement of surrounding cells.

30

structure hosting stem cells for corneal epithelium?

palisade of Vogt

31

what is the XYZ hypothesis of corneal health?

X is proliferation and stratification at limbus, Y is centripetal migration of epithelial cells centrally, Z is desquamation of old corneal cells. For K to be healthy X +Y >Z

32

triad of KPs, elevated IOP, and corneal edema indicates what?

HSV

33

cyclosporins are associated with what side effect?

disc edema

34

what side effect is associated with Etanercept and Infliximab

optic neuritis

35

Kpro biggest two down sides?

risk of glaucoma and endophthalmitis

36

severe atopic dermatitis and keratoconjunctivitis can be treated with what oral med LONG term?

tacrolimus

37

chronic cicatricial conjunctivitis signs? differential?

fornix fibrosis and shortening.
infection (adeno, trachoma), mucus membrane pemphegoid, trauma/chemical injury, neoplasm

38

features of vernal conjunctivitis

<10 y/o M, giant papilla, horner-trantas dots (limbal follicles)

39

topiramate is associated with glaucoma. what are the 3 primary treatments?

stop topiramate, topical hypotensives, cycloplegia (for often associated mild AC raection)

40

azithromycin has what side effect?

QT prolongation

41

how to treat gonococcal conjunctivitis? what if there's corneal involvement?

conjunctivitis 1g ceftriaxone IM.
if there's corneal involvement--admit pt for IV ceftriaxone

42

what is specular microscopy?

confocal microscopy live imaging of endothelium.

43

whats the normal density of endothelial cells

1500-3500 cells/mm2

44

what is confocal microscopy of the cornea good for?

in vivo analysis of the 5 layers. can identify causative organisms of infection, corneal nerve morphology, eval of each layer in dystrophies

45

what are the 4 main zones of the cornea?

central, paracentral, peripheral, limbal

46

what are the three key components giving cornea its optical properties

shape, curvature, power

47

what is the optical zone of the cornea

cornea overlying pupil

48

what is cornea apex vs cornea vertex?

apex is the point of max curvature, vertex is point at intersection of patient's line of fixation and corneal surface.

49

whats the average refractive power of the cornea?

+43D; +49D from anterior surface -6D of posterior surface

50

what is keratometry and what does it measure? limitation?

measurement of power of central cornea. It calculates radius of curvature in mm.

only measures anterior power of central cornea

51

what is keratoscopy?

projection of placido rings to provide qualitative information of the entire anterior cornea

52

what is topography vs keratoscopy?

topography is digitally projected and represented with colors

53

bow-tie pattern on topography?

astigmatism

54

crab claw configuration on topography is classic for what? what else is on differential?

pellucid marginal degeneration, or keratoconus is also ddx.

55

what is angle kappa? what is a desired value when calculating IOLs

angle between visual axis and pupillary axis (optical axis). want it to be <0.4mm

56

what should you avoid if angle kappa is >0.4?

multifocal IOLs. given line of sight and pupillary axis (optical axis) are far apart

57

what is corneal tomography vs topography? what technology is tomography based on ?

tomography can capture 3D structures of the cornea--anterior and posterior curvature, corneal thickness, AC depth, lens information.

-placido and scanning slit

58

what filter is used to see fluorescein

cobalt blue

59

what is dye disappearance test?

timing duration of fluorescein presence --if prolonged there may be nasal lacrimal system blockage.

60

punctate staining at 3 and 9 o'clock is classic for what cause

due to CL wear

61

what is the basic secretion test?

filter strip 5mm wide 30 mm long, in fornix with anesthetic--to measure basic tear production; <3mm in 5 mins is abnormal

62

what is schirmer I test--how is it done and what does it test for? what's abnormal

filter strip to fornix withOUT anesthetic to test for basic AND reflex tearing. <5.5mm in 5 mins is Abnl.

63

what is schirmer I test--how is it done and what does it test for? what's abnormal

filter strip to fornix withOUT anesthetic + nasal mucosa irritation with cotton tip. <15 mm in 5 mins is Abel

64

comment on specificity and sensitivity of schirmer tests

specific but NOT sensitive

65

whats the normal osmolality of tear

306-308

66

what are some markers of dry eye that we can assay for ?

lactoferrin, IgE (for allergic), matrix metalloproteinase 9 (MM9) >40

67

what is corneal esthesiometry and how is it done?

corneal sensation test--> cotton whisp without anesthesia, before IOP check, and tested in all 4 quadrants

There's also a handheld esthesiometer (cochet-Bonnet)

68

what is corneal hysteresis

the difference in pressure of the cornea bending inward vs bending outwards during air jet applanation

69

What is corneal resistance factor?

correction between corneal hysteresis and thickness.

70

terrien marginal degeneration classic features?

marginal corneal thinning with lipid keratopathy in 4-5th decade of life resulting in against the rule, oblique, or irregular astigmatism.

71

patient population for floppy eye lid

obese with OSA

72

underlying problem with floppy eyelid

lax upper tarsus

73

recurrent corneal erosions. what are 3 causes?

past trauma, past HSV, epithelial dystrophy

74

treatment options for recurrent erosions?

lubricate/hypertoic saline, BCL, epithelial debridement vs anterior stromal puncture, PTK vs PRK

75

what is distichiasis?

two rows of eye lashes (second coming out of meibomian glands

76

what is mucus fishing syndrome

patients fixated on fishing strands of mucus out of eye and eye rubbing leading to more mucus production. +lissamine green and rose bengal inferior K and conj

77

what is congenital anterior staphyloma?

opaque cornea that is partially or completely absent of decemet's and endothelium and lined with uveal tissue posteriorly to cornea causing iris problems. usually unilateral

78

what is keratectasia and how is it different than congenital anterior staphyloma?

keratectasia is opaque cornea without decemet's or endothelium. Anterior staphyloma has also a layer of underlying uveal tissue that keratectasia does not.

79

which IOP lowering drop is associated with reactivation of HSV keratitis?

prostaglandin analogues

80

microcornea dimensions? mode of inheritance? px?

<10 mm; autosomal dominant. px generally well with glasses

81

megalocornea dimensions? mode of inheritance

>13mm; sex linked recessive

82

megalocornea associated gene?

CHRDL1

83

megalocornea associated abnormalities?

diaphany, goniodysgenesis, ectopia lentes, arcus, zonule instability, glaucoma (not congenital glaucoma.

84

dimensions of cornea plana and sclerocornea?

<43 D

85

Cornea plana is associated with what gene?

KERA(12q22)

86

ocular associations of cornea plana?

close angle glaucoma, cataract, coloboma, hyperopia

87

Finnish ancestry is associated with what disease

cornea plana

88

3 waves of neurocrest cell migration at 6 weeks make up what structures

K endothelium, stroma, and iris

89

mode of inheritance for sclerocornea?

sporadic is most common.

90

manifestation of syphilic intrauterine keratitis?

typically 6-12 years old; sudden onset K edema, vascularization formation of salmon patch that regresses to leave ghost vessels

91

Association of Lowe's syndrome?

oculocerebrorenal. cataract, kidney disease, and corneal keloid

92

corneal keloids are seen with what conditions?

Lowe's, Rubinstein-Taybi, ACL (acromegaly, cutis vertices gyrata, corneal leucoma syndrome)

93

forceps birth trauma is associated with what eye findings

Horner's and Vertical Haab's striae.

94

What is congenital corneal anesthesia?

bilateral sterile ulcers and PAINELESS K opacities as infant or child.

95

what's the difference between degeneration and dystrphy

dystrophies has inheritance pattern

96

deceit's thickness at birth and in elderly?

3 microns -->13 microns

97

what are age related Guttate called?

Hassall-Henle bodies; due to loss of endothelial cells.

98

What are coat's white ring

residual subepi/stromal haze after metal FB. It won't change after acute inflammation has resolved...so no use for steroid at this point

99

what is Spheroid degeneration

Either primary (degenerative) or secondary (due to inflammation)-- translucent subepi, bowman's or superficial epithelial deposits. Looks oily. Mostly on cornea but can involve conj

100

other names of spheroid degeneration?

Laborador keratopathy, Bietti nodular dystrophy, actinic keratopathy, climatic droplet keratopathy

101

causes of BK?

inflammation, hereditary, hyperphos, hypercalc, hyperurecemia, mercury vapor exposure

102

How to treat BK?

EDTA chelation

103

Ferry line?

K iron line from bleb

104

Fleisher Ring?

iron line at base of keratoconus

105

Hudson-Stahli line

WNL due to aging; at junction of upper 2/3 and lower 1/3 of K

106

Stocker line

pterygium

107

LASIK line

at margin of ablation zone or more centrally

108

Chrysiasis

Gold deposition in cornea; often peripheral

109

Chalcosis

copper deposition in K due to copper FB

110

Argyriasis

silver deposition in cornea

111

Arcus is due to lipid deposition where?

both bowman's and decemets

112

White Limbal girdle of Vogt?

Type I: early BK with clear zone between limbus
Type II: no clear zone between limbus; more punctate in older individuals

113

what is corneal farinata? mode of transmission

reflective material in keratinocytes of deep stromal layer giving a dot or common like shape on retroillumination; dominant

114

crocodile shagreen is also called what

mosaic degeneration

115

what is senile furrow degeneration? what should you be careful of?

apparent or real thinning in the area of senile arcus. Cat surgeons should take caution to prevent wound leak

116

Terrien marginal degeneration- what is it? what age are patients? where does it start? complications of it?

peripheral thinning of the corneal without epithelial loss. Age >40. Starts Supranasally and spreads. Complications include astigmatism (usually against the rule and oblique) can cause spontaneous perforation.

117

Terien marginal degeneration in a young person with inflammation... you should think?

Fuch's superficial marginal keratopathy

118

what is lipid keratopathy?

deposition of cholesterol, fats, and glycoproteins s/p prolonged K inflammation--> leading to scarring and vascularization.

119

What condition looks like lipid keratopathy?

Schnyder corneal dystrophy (autosomal dominant stromal dystrophy), bilat opacification from lipid and cholesterol accumulation.

120

what are Cogan plaques?

calcium deposition along scleral collagen fibers anterior to horizontal rectus muscle insertion

121

If vision is decreased when on amiodarone or tamoxifen what should you consider?

optic neuropathy... given that verticillate is unlikely to reduce vision

122

What are some meds associated with verticillate? what systemic condition?

amiodarone, indomethacin/ibuprofen, chloroquine/hydroxychloroquin, tamoxifen, silver, gold, antacids, gentamicin, clarithromycin

123

Side effect of Cytarabine?

inhibition of DNA synthesis can cause painful epithelial cysts and punctate keratopathy-- and KEDs

124

what kind of depositions are associated with ciprofloxacin?

chalky white crystalline deposition in KEDs

125

What depositions are seen with tetracyclines?

adrenocrhome (breakdown of epinephrine) dark spots in K or conj

126

rifabutin can deposit where?

endothelium

127

what are the 4 categories of corneal dystrophies?

1. epithelial sub epithelial
2. Epithelial stroma
3. stromal
4. endothelial

128

what pathogens can invade through intact corneal epithelium?

neisseria, listeria, corynebacterium

129

conditions associated with limbal stem cell deficiency?

chemical burns, SJS, GVHD, aniridia

130

honey comb subepithelial opacities are associated with what?

Thiel-Behnke dystrophy

131

Granular dystrophy histologic features?

hyaline deposition in stroma-- red staining with Masson trichrome

132

zoster immunization decreases likelihood of zoster by how much?

50%

133

what are the histological features of mapdot fingerprint/EBMD?

sheets of intraepithelial basal lamina material (map)
intral epithelial extension of basal laminar material (fingerprint)
intraepithelial psuedocysts containing cytoplasm (dots)
irregular subepi of fibrogranular material (bleb)

134

other names of map dot finger print (4)

endothelial basement membrane dystrophy. cogan microcystic epithelial dystrophy, anterior basement membrane dystrophy

135

Meesman epithelial corneal dystrophy (MECD) clinicalfeatures? mode of transmission?

small intraepi vesicles on regroillumination. can have whorled and wedge shaped epithelial pattern, slightly thinned cornea, decreased corneal sensation. Dominant.

136

What is the histological feature of meesman epithelial dystrophy?

PAS positiveepitheilal cells with dense fibrogranular material surrouded by tangles of cytoplasmic filaments "peculiar substance". thickened basement membrane and frequent mytosis

137

What is Stocker-Holt?

earlier onset variant of Meeseman

138

What are clinical features of Lisch epithelial corneal dystrophy? Mode of inheritance?

band shaped, feathery gray lesions with whorled/flamed pattern. Also has microcysts in epithelium. More isolated to areas of the cornea. Usually is painless. X linked dominant.

139

Hystological features of Lisch epithelial corneal dystrophy?

PAS staining in cytoplasmic vacuoles. Ki67 staining of mitotic activity.

140

Gelatinous droplike corneal (epithelial) dystrophy. Other names? mode of inheritance?

subepithelial amyloidosis. primary familial amyloidosis. Autosomal rescessive.

141

gelatinous droplike corneal dystrophy. clinical presentation?

mull burry like small nodule subepi lesions--decreased vision, photophobia, irritation, tearing, protrusion of lesions, larger "kumquat-like lesions". Second decade of life.

142

TGFB1 gene is at what locus? responsible for what protein?

5q31-- kertoepithelin

143

what are the two bowman's dystrophies? what gene is affected?

reis-buckler and thiel behnke. TGFB1 on 5q31 affecting keratoepithelin

144

what are the histological features of Reis-Buckler?

sheets of granular deposits that stain red on Masson trichrome. Immunopositive for TGFb1

145

Clinical features of reis buckler dystrophy? age of onset?

irregular/coarse geographic opacities in bowman's layer. onset first few years of life

146

clinical differences between Reis bucker and Thiel Behnke?

Reis bucker is more severe but Thiel Behnke recurs more.

147

histological feature of Thiel Behnke?

saw tooth fibrocellular material in bowman's layer. --positive immunostaining for TGFB1

148

clinical feautres of Thiel Behnke?

first second decade of life starting as solitary flecks of honeycomb pattern sparing peripheral cornea.

149

What is another name for Waardenburg-Jonker corneal dystrophy?

Thiel Behnke

150

Histological feature of lattice corneal dystrophy Type 1 (classic)

arborizing amyloid deposits in anterior stroma. focal thinning/absence of Bowman's layer.
-red with congo red with apple green birefrengence.

151

What are two unrelated conditinons that may look like lattice dystrophy?

fungal hyphae; monoclonal gammopathy

152

Granular corneal dystrophy type 1 histological features?

granular hyaline deposition with Masson Trichrome. also stains for TGFb1

153

bread crumb like appearance of cornea

Granular stromal dystrophy.

154

clinical feautres of granular dystrophy classic type 1?time of onset? px?

breadcrumb appearance, vacuoles, glassy splinter appearance. slowly progressive. first couple decades of life onset. px generally good

155

What is Avellino corneal dystrophy. histological features?

Granular dystrophy type II-- it has both granular dystrophy's hyaline depositis and amyloid deposits of lattice dystrophy

156

Clinical features of Avellino corneal dystrophy?

snow-flake like "icicle" like corneal appeareance with deeper stromal lattice lines.

157

what late stage occurence in keratoconus leads to acute hydrops?

decemet breaks

158

conditions associated with keratoconus?

atopic disease, Downsyndrome, osteogenesis imperfecta, sleep apnea, MVP, Ehler Danlos, vernal keratoconj, retinitis pigmentosa, floppy eyelids

159

Macular corneal dystrophy involves what layers? age of onset?

can involve all the layers. between 3-9 y/o.

160

Clinical features of macular corneal dystrophy?

superficial, irregular, white fleck-like opacities with focal gray-white superficial stromal opacities with INTERVENING HAAZE. can involve all layers of cornea. Hypoesthesia can be seen.

161

How to diagnose macular dystrophy aside from clinical feautures?

ELISA against keratan sulfate

162

What stains are used for Schnyder corneal dystrophy?

oil red O, sudan black. Tissue must be sent FRESH.

163

What lab test should you get in Schnyder corneal dystrophy?

fasting lipid panel. dietary modification is recommended but does not lead to better px

164

Congenital stromal corneal dystrophy. Presentation?

congenital diffuse bilateral clouding--stromal flake like white opacities. thickened cornea

165

Fleck corneal dystrophy. pathology? is it progressive?

excess glycosaminoglycan--stains alcian blue and colloidal iron. Also stains lipids-sudan black and oil red O. It is NOT progressive.

166

microscopy features of Posterior amorphous corneal dystrophy? Is it progressive? Gene association?

attenuated endothelial cells. Irregular stroma anterior to decemets. Nonprogressive. KERA gene--thus associated with cornea plana, hyperopia.

167

PreDecemet corneal dystrophy. Associated with what disease?

X linked icthyosis

168

PreDecemet corneal dystrophy. microscopy features? clinical features?

large keratocytes in posterior stroma. vacuoles and intracytoplasmic inclusions. >30 y/o==fine polymorphic gray opacities.

169

Three stages of Fuch's endothelial dystrophy?

1: guttate that spreads from central out
2: endothelial decompensation and stromal edema
3: bullous keratopathy

170

Treatment options for Fuch's?

mild disease: Murad (NaCl), BCL, IOP lowering. Anterior stromal puncture, amniotic membrane.
Severe: PK vs endothelial transplants.
Novel: intracameral Rho Kinase (ROCK) inhibitor to stimulate endothelial proliferation

171

Posterior polymorphous dystrophy is also called what?

Schlichtung dystrophy.

172

Posterior polymorphous dystrophy. clinical presentations?

endothelial vesicles, geographic gray lesions, endothelial bands with scalloped edges. Stromal edema, corectopia, iridocorneal adhesions.

173

Posterior polymorphous dystrophy. pathological features?

multiple layers of endothelial cells that behave like epithelial cells--have microvilli, keratin, rapid growth, desmosomes.

174

Congenital hereditary endothelial dystrophy (CHED). clinical features? path features?

clinical: blurry vision, nystagmus withOUT tearing/photophobia. diffuse ground glass haze.
Path: thickened decemets and sparse atrophic corneal endothelial cells.

175

what condition is associated with increased inferior to superior power ratio of the cornea?

keratoconus.

176

best imaging options for keratoconus?

Scheimflug imaging, anterior OCT. --Showing decentered bow tie

177

keratoectasia is associated with what surgery?

LASIK and PRK

178

is hydrops an indication for emergency PK?

no

179

Pellucid marginal degeneration (PMD) --laterality and hereditary pattern? what is it?

bilateral, nonhereditary. non inflammatory.
Peripheral thinning

180

crab claw pattern of cornea thinning

pellucid marginal degeneration.

181

Px of pellucid marginal degeneration vs keratoconus?

PMD is worse because any transplant will be closer to the limbus and more prone to rejection

182

Keratoglobus. laterality, age of onset?

bilateral, noninflammatory, and typically present at birth

183

Keratoglobus is associated with what physical exam finding and condition?

blue sclera. Ehler Danlos

184

where is it think for keratoglobus?

paracentral inferior-decemetc, bowmans, and stromal thinning.

185

keartoglobus treatment?

CL, PK, tectonic lamellar keratoplasty followed by PK.

186

Kayser Fleischer ring found in what diseases?

Wilson's primary biliary cirrhosis, chronic hepatitis, exogenous chalcosis.

187

of all the mucopolysaccharidosis conditions... all of them are what inheritance pattern? except for 1 which is what? and how is it transmitted?

all are autosomal recessive except for Hunter's which is X linked recessive.

188

what are the three varieties of Mucopolysaccharidosis I disorders?

Hurler, Scheie, Hurler-Scheie.

189

Hurler syndrome... what is the missing enzyme

a-L-iduronidase.

190

ocular features of Hurler/ Scheie syndrome?

corneal opacity, optic atrophy, retinopathy.

191

what's the difference between Hurler and Scheie?

they are the same disorder except for Hurler is severe with additional intellectual impairment and short lifespan vs Scheie is later onset (age 5-15) and have normal intelligence and life span.
Hurler can have pigmentary retinopathy, glaucoma, optic nerve swelling/atrophy, hypertelorism

192

How to diagnose Hurler/Scheie?

leukocyte/ plasma enzyme study. urine analysis, conj biopsy (rarely used)

193

what is the accumulated material in MPS I (hurler's/ scheie)

dermatan and heparan sulfate

194

what is MPS II disorder?

Hunter's

195

Hunter's syndrome enzyme? accumulate?

Iduronidase 2 sulfatase, dermatan and heparan sulfate

196

Hunter's features?

exophthalmos, hyperteloris, optic nerve swelling/atrophy, retinopathy

197

What is Sanfilippo syndrome?

MPS III associated with pigmentary retinopathy, abnormal ERG.

198

Morquio syndrome--clinical features?

shallow orbits, retinopathy, mild K opacity in 10% of patients >10 years old

199

Morquio syndrome--what is the pathophys?

MPS IV--missing galactose 6 sulfatase (A) and b-galatosidase (B) accumulate of keratin sulfate (A) and chondroitin sulfate (B).

200

Maroteaux-Lamy syndrome--pathophys?

MPS VI. missing N acetylgalactosamine-4 sulfatase; accumulate dermatan sulfate.

201

Maroteau-Lamy clinical features?

severe K clouding within 1 year of life. K edema. Also narrow angle glaucoma and optic nerve atrophy

202

Sly syndrome -clinical features?

corneal clouding

203

Sly syndrome- pathophys?

MPS VII. beta glucuronidase deficiency. dermatan sulfate, keratan sulfate, chondroitin sulfate accumulation.

204

Natowicz syndrome--pathophys/ clinical features

MPS IX. hyaluronidase 1 missing. accumulation of chondroitin sulfate. no corneal clouding

205

how is px of PK for patients with K clouding due to mucopolysaccharidosis

guarded as can accumulate in graft

206

How many sphingolipidosis conditions are there...what are they

4.
Fabry's, Tay Sachs, multiple sulfatase deficiency, generalized gangliosiosis

207

What are the organisms that can invade intact cornea

Shigella, H flu biotype III, Neissieria (both gonorrhea and meningitidis, Listeria, fusarium, and corynebacterium

208

which bacteria have exotoxin that can induce corneal cell necrosis

pseudomonas, strep, and staph

209

sphingolipidosis --corneal finding. other ocular findings

verticillata in epithelial layer. periorbital edema 1/4th of cases, posterior spokes like cataracts 50%, conj aneurysms (60%), papilledema, retinal swelling, optic atrophy, retinal vascular dilation

210

Fabry's extraocular findings

renal failure, peripheral neuropathy, angiokeratomas,

211

multiple sulfatase deficiency extra ocular findings

metachromic leukodystrophy, progressive psychomotor decline. usually die within 1 year.

212

unilateral arcus or asymmetric arcus is likely due to what

carotid atherosclerosis

213

sabourad agar cultures what

fungi

214

Lowenstein jensen agar cultures what

mycobacterium

215

thioglycolate agar cultures what

aerobes and anaerobes

216

name the 3 lysosomal storage diseases?

Fucosidosis, mannosidosis, Goldberg syndrome.

217

What is the function of LCAT (non ophthalmology)

LCAT transports excess cholesterol from peripheral tissue to liver

218

What are LCAT deficiency associations--systemic? ocular?

systemic: renal insufficiency, atheroscolerosis.
Ocular: arcus, "nebular corneal clouding

219

What is fish eye disease?

variant of LCAT deficiency. --corneal clouding, arcus

220

which chromosome abnormality is associated with fish eye disease and LCAT deficiency.

16q22.1

221

Tangier disease pathophys? genetic locus? exam findings?

missing serum high density alpha lipoproteins. disease maps to 9q22. large orange tonsils, spleen and LN

222

Crystalline keratopathy occurs in immunocompromised corneas. what's that most likely causative agent?

viridans strep--branching colonies within corneal stroma

223

what is a complication of congenital cataract surgery that could present at any point in the patient's life?

glaucoma--especially with young patients, 3 months post op, small K diameters

224

TIGR/MYOC gene is associated with what?

junvenile open angle glaucoma

225

OPTN gene is associated with what?

normotension glaucoma

226

CYP1B1 gene is associated what what

congenital glaucoma

227

LOX1 is associated what what

pseudoexfoliation

228

treatment regimen for burn injuries

ppx abx, cycloplegic, steroid-- debridement if needed

229

transient corneal edema due to cold exposure can occur in which conditions?

Raynaud's and CN V dysfunction

230

time course of UV radiation exposure?

Snow vs. welding vs. sun lamp. asymptomatic initially then pain few hours later as epithelium sloughs off. Usually self limited. Can use ointment and cycloplegia

231

cystinosis eye finding

polychromatic cysteine crystals in conj, cornea, iris

232

eye treatment for cystinosis?

cysteamine eye drop to convert cystine to a disulfide resembling lysine

233

tyrosinemia corneal findings? derm findings?

cornea will have pseudodendritic lesions and corneal erosions; derm is hyperkeratotic lesions of the palm/soles

234

treating tyrosinemia?

dietary restriction of tyrosine and phenylalanine

235

ocular manifestation of alkaptonuria?

pigment deposition in bowman's layer and epithelium. ochronosis due to excess homogentisic acid--treat with vitamin C to decrease arthritis

236

Meretoja syndrome/Gelsolin type lattice corneal dystrophy

amyloid deposits, dermatochalasis, lagophthalmos, pendulous ears, lattice lines

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porphyria eye findings

necrotizing scleritis, corneal thinning

238

Ehlers Danlos type VI. eye findings. systemic findings

easy ruptured/brittle cornea, blue sclera, keratoconus, keratoglobus. scoliosis and moderately joint/skin distensibility

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locus of fibrillar gene

15q21

240

osteogenesis imperfecta. gene associated? type I vs Types 2-4? ocular findings?

COLIA1, COLIA2; Type I blue sclera fades with time; other findings include optic nerve damage due to fx, keratoconus, megalocornea

241

vitamin A deficiency causes what?

night blindness(decreased rhodopsin), xerophthalmia (loss of goblet cells)--bitot spots

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what patients get vitamin A deficiency?

babies that are malnourished with stressors such as diarrhea and measles.
Adults with chronic alcoholism, lipid malabsorption

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px of vitamin A deficiency

50% mortality rate untreated

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ocular signs of monoclonal gamopathies (MM, waldenstrom, cryoglobulinemia)

crystalline deposition in cornea, copper in cornea, slugging of blood flow, pas plant proteinaceous cysts, infiltrate of sclera, orbital bony invasion with proptosis

245

what are vertical lines in decemet's and deeps stroma called in DM?

Waite-Beetham lines

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differences between ichthyosis vulgaris and x linked ichthyosis

x linked up to 50 % may have corneal opacities vs ichthyosis vulgaris usually does not. Both have eye lid scales, iccatricial ectropion

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acute hemorrhagic conjunctivitis are usually caused by what?

entero virus or coxackie virus

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Crystalline deposits occurs with:

myloidosis as in multiple myeloma;
cystinosis;
treatment with gold;
uric acid from uraemia

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Pterygium:

>fibrovascular overgrowth of the bulbar conjunctiva
>the stroma shows basophilic degeneration (elastotic) of collagen
>the epithelium is often thin but may show hyperplasia or dysplasia
>it is not regarded as precancerous

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Keratoconjunctivitis sicca (dry eyes)

>causes dysplasia of the epithelium which may eventually become keratinise
>the goblet cells may increase due to chronic irritation from dry eyes
>in Sjogren's syndrome, there is lymphocytic infiltration of the lacrimal and accessory glands

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Merkel cell carcinoma:

>an aggressive primary tumour of the skin, arising from papillary dermis
>usually presents as a firm non-tender solitary skin nodule on the face and neck
>early metastasis through the lymphatic channels
>contains APUD (amine precursor and uptake decarboxylation) cells