631 - 3 - Corneal Dysgeneses And Dystrophies, Ocular Dz

Front 1

"dysgenesis" is hereditary, congenital, or neither?

Back 1

congenital, but may have a hereditary pattern.

Front 2

what is characterized by a severe decrease in the corneal curvature, and therefore a shallow anterior chamber?

Back 2

cornea plana

Front 3

what is a condition in which the adult corneal diameter is <10 mm

Back 3

microcornea

Front 4

what type of refractive error is microcornea usually associated with?

Back 4

hyperopia, the K's at birth can be around 50

Front 5

what is a risk factor of microcornea due to the crowding of the AC.

Back 5

glaucoma.

Front 6

at what age is the cornea usually at adult size? and what is the normal in mm.

Back 6

by age 3-4 and they should be 11mm - 12mm. <10 is microcornea.

Front 7

what is the normal mm diameter for a neonates cornea?

Back 7

9-10mm < 10 is microcornea

Front 8

what is more common micro cornea or megalocornea?

Back 8

megalocornea.

Front 9

cornea diameter >13mm is indicative of what condition.

Back 9

megalocornea

Front 10

who is affected more with megalocornea men or women and why?

Back 10

men b/c it is usually X linked recessive. but it may be autosomal dominate.

Front 11

what refractive error is expected in megalocornea?

Back 11

high myopia and astigmatism.

Front 12

what is a critical ddx with megalocornea?

Back 12

congenital glaucoma- refer these pts to the glaucoma specialist

Front 13

what is rare, resembles megalocornea, but here the cornea is very thin especially in the periphery.

Back 13

keratoglobus. this could also be classified in the dystrophies.

Front 14

what diseases are classified as dysgeneses, and have abnormalities of size or curvature

Back 14

cornea plana, microcornea, megalocornea, keratoglobus.

Front 15

what two diseases are classified as mesenchymal dysgeneses.

Back 15

posterior keratoconus, and peter's anomaly.

Front 16

is posterior keratoconus related to anterior keratoconus?

Back 16

no

Front 17

is the anterior surface normal or abnormal with posterior keratoconus? if so how?

Back 17

nope its normal

Front 18

what type of acuities would you expect with posterior keratoconus?

Back 18

near normal

Front 19

what consists of an indentation of the posterior cornea that works its way forward and variable degrees of central stromal hazing?

Back 19

posterior keratoconus

Front 20

what anomoly is associated with a kerato-lenticular adhesion?

Back 20

peter's Anamoly.

Front 21

pupilarry ruff adhesion to the posterior cornea with a secondary glaucoma as a possibility as well as opacification of the cornea is what?

Back 21

peter's anomaly

Front 22

Name three anterior chamber cleavage syndromes

Back 22

posterior embryotoxon
axenfeld's anomaly or syndrome
riegers anomaly or syndrome

Front 23

list the structures from the most anterior to posterior as seen in a gonio lens

Back 23

schwalbes line
trabecular mesh
scleral spur
ciliary body
iris

Front 24

anterior displacement of schawlbes line is part of what?

Back 24

posterior embryotoxon

Front 25

if you wanted to look for posterior embryotoxon what position (on the clock) would you look at.

Back 25

outer edge of the cornea at 3 and 9 oclock

Front 26

If a patient has an anterior displaced schwalbes line as well as iris strands, this is known as what?

Back 26

Axenfeld's Anomaly or syndrome

Front 27

what is the difference b/t Axenfeld's Anomaly and sydrome

Back 27

Anomaly has normal IOP
syndrome has elevated IOP

Front 28

what is Rieger's anomaly and syndrome?

Back 28

anteriorly displaced schwalbes line
iris strands
iris hypoplasia (not fully developed)
often corectopia (distorted iris)
anomaly involves just the eye
syndrome involves systemic findings as well such as (primarily) facial hypoplasia (missing teeth)

Front 29

in general what hereditary pattern do corneal dystrophies show?

Back 29

usually autosomal dominant

Front 30

dystrophies are generally bilateral or unilateral

Back 30

bilateral

Front 31

in general when is the onset of dystrophies and how do they proceed? lastly where on the cornea do they generally occur

Back 31

onset early in life (by second decade)
generally progress slowly
generally involve a single layer centrally

Front 32

what are the 2 epithelial layer dystrophies that we talked about?

Back 32

epithelial basement membrane dystrophy and meesmans juvenile epithelial dystrophy

Front 33

epithelial basement membrane dystrophy is also known as? three more names.

Back 33

map dot fingerprint
cogans microcystic dystrophy
anterior basement membrane dystrophy

Front 34

what is the most common anterior corneal dystrophy?

Back 34

epithelial basement membane dystrophy. (EBMD)

Front 35

what are some of the symptoms associated with EBMD, mild, moderate, and severe?

Back 35

mild: asymptomatic, maybe short lived irritation in the mornings from lids lifting epi off. Moderate: chronic corneal irritation may wake them up, photophobia, trasient visual fluctuations. Severe: HX of recurrent spontaneous corneal erosions, reduction in va

Front 36

objectively what will you see with an EBMD pt?

Back 36

intraepithelial microcysts and sub epi lines best seen with retro illumination. and a TBUT that breaks up instantly

Front 37

what are maps and will the "maps" stain in EBMD?

Back 37

they are gray patches with sharp margin, geographic lines or patterns that become more pronounced with age. they will stain negatively.

Front 38

what are dots in EBMD? color, shape, arrangement size...

Back 38

discrete grayish clear spots,, that are round or comma shaped and can be clustered and <1mm. they are often seen with maps.

Front 39

fingerprints may also be seen with EBMD. what are they?

Back 39

large patterns centrally that are swirled refractile lines like a fingerprint.

Front 40

what is the most common complication with EBMD? and what can it lead to?

Back 40

spontaneous epithelial erosions, can lead to pain, tearing, photophobia, perilimbl injection, FB sensation, increased risk of infection, scarring and vision loss.

Front 41

what is the treatment for mild EBMD

Back 41

hypertonic 5% NaCl ung at night and lubrication as needed.

Front 42

what is the treatment for moderate EBMD?

Back 42

hypertonic solution q3 - 4 h
hypertonic ointment qhs
high water content cl TIGHT FITTING
homatropine
prophylactic antibiotic qid if epi is compromised
diclofenac .1% (nsaid)
lid hygiene

Front 43

Tx for sever EBMD

Back 43

instil anesthetic and debride loose epi around defect then add a SCL to wear until epi has grown back.
hypertonic saline q2-3hs
propholactic antibiotic until re-epi
when it wont heal refere to corneal spec. or perform anterior stromal puncture.

Front 44

with moderate EBMD how long should you use the hypertonics and or SCL?

Back 44

for several weeks after symptoms have subsided.

Front 45

what is the pt education on EBMD?

Back 45

good lid hygiene, and there is no cure for this and it is recurrent in nature.

Front 46

why is Meesmans juvenile epithelial dystrophy an exception to most other dystrophies?

Back 46

dystrophies usually occur in the central cornea this doesnt its in the periphery

Front 47

you see some intraepithelial spots with retro illumination in the periphery of the cornea. The pt may also have some areas of epithelial erosions after 40 years of age. what is this

Back 47

meesmans juvenile epithelial dystrophy. usually begins within the 1-2 years of life.

Front 48

what is a visual acuity problem that can occur due to meesmans juvenile epi dystrophy

Back 48

irregular astigmatism. this is the dystrophy that has intraepithelial spots in the periphery.

Front 49

name the 2 bowmans layer dystrophies we talked about?

Back 49

reis bucklers dystrophy and anterior mosaic dystrophy.

Front 50

you see a gray white irregular opaque pattern in bowmans layer that looks like a fish net. what could this be?

Back 50

Reis bucklers dystrophy. (a fisherman named reis with a buckle and a net on the bow.

Front 51

what are some of the complications with reis bucklers dystrophy?

Back 51

painful RCE, anterior stromal opacification, VA drop by age 20-30

Front 52

TX for reis buckler dystrophy?

Back 52

erosion tx
low vision
keratoplasty

Front 53

in bowmans layer you see gray polygonal opacities that is not affecting the pts vision but is central. what could it be

Back 53

anterior mosaic dystrophy

Front 54

three anterior stromal dystrophies are?

Back 54

granular dystrophyn (most common)
lattice dystrophy
crystallin dystrophy

Front 55

10 year old pt you see central discrete white bread crumb or snowflake like opacities in the anterior stroma. VA could be affected what is it?

Back 55

granular dystrophy.

Front 56

what is the treatment for granular dystrophy

Back 56

keratoplasty
low vision

Front 57

in the stroma of a pt you see refractile branching lines with white dots and central haze that appears like ground glass. what is it

Back 57

lattice dystrophy

Front 58

what are some of the complications associated with lattice dysrophy

Back 58

RCE

Front 59

what is the Tx for lattice dystrophy

Back 59

tx the RCE, low vision aids, keratoplasy by age 40

Front 60

what forms yellow/white crystals in the first to second decade? what are the crystals made of?

Back 60

crystalline dystrophy, composed of cholesterol.

Front 61

what is a patient that has crystalline dystrophy at risk of developing by the age of 30 or 40?

Back 61

arcus b/c of the accumulation of cholesterol deposits.

Front 62

what do you do for a pt with crystalline dystrophy

Back 62

the only thing mentioned in class is to refer them for a cardio vascular workup.

Front 63

what procedure is used to clear the central epi and anterior stromal corneal dystrophies and is less invasive than pkp?

Back 63

phototherapeutic keratectomy

Front 64

diffuse corneal clouding by age 5 -9 due to the stroma being invaded by mucopolysaccharide material.

Back 64

full thickness stromal: macular dystrophy.

Front 65

is full thickness stromal: macular dystrophy autosomal recessive or dominant?

Back 65

this is an exception compared to most dystrophies. it is autosomal recessive.

Front 66

a pt with thinning or the cornea <500um, nonsymmetrical curvature of the cornea, and bulging. what do they have

Back 66

ectatic dystrophy: keratoconus

Front 67

give a general timeline on how keratoconus progresses

Back 67

usually starts around age 15 to 25 and progresses for 5 to 6 years, stabilizes, but then it may destabilize 10 to 15 years later.

Front 68

secondary complication with keratoconus include

Back 68

glare, photophobia, and possibly diplopia.

Front 69

while performing ret you see a scissor effect, then while performing opthalmoscopy you see an oil drop appearance what is this commonly seen in?

Back 69

keratoconus

Front 70

what is better for a pt with keratoconus contacts or specs

Back 70

cls give better VA's.

Front 71

central to inferior central bulging of the cornea is known as what sign?

Back 71

munsons sign

Front 72

what is the name of the iron ring deposit at the base of a keratoconic cornea?

Back 72

fleischers ring

Front 73

what are the lines called that you see at the posterior surface of the cornea in a pt with keratoconus?

Back 73

vogt's striate

Front 74

complication with edema are possible with keratoconus why?

Back 74

hydrops can form b/c the endothelium breaks and descemets membrane splits.

Front 75

what must keratoconus be DDx from

Back 75

keratoglobus and posterior keratoconus