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What cells is neuroblastoma derived from?

Precursor cells for the sympathetic pathway. Therefore it excretes catacholamines

1

What are racoon eyes a sign of?

Neuroblastoma

2

Congenital horners should raise suspicion for

Neuroblastoma bc it messes w the sympathetic pathway
Check urine catacholamines

Symp dilates
Therefore miosis

3

What do eyes look like in primary position in browns syndrome?

hypotropia of effected eye

4

Stumped

Sclerocornea
Trauma (foreceps)
Ulcer
Mucopoly
Peters
Endothel dystrophy (ched 2 AR cloudy from birth)
Dermoid

5

on eom in browns what do you see

unable to elevate when adducted improves upon abduction

6

how do you treat duanes surgically?

recess the (tight) medial rectus of effected eye

7

how do you surgicaly treat upshoots and downshoots in duanes?

Y split of lateral rectus of effected muscle

8

objects that fall on the horopter stimulate what?

corresponding retinal points

9

whats kassebach-merrit syndrome

in capillary hemangioma there may be plt sequestration causing thrombocytopenia. so check cbc in kid w cap hemangioma

10

diplopia in XT is crossed or uncrossed?

crossed (stimulates temporal retinas)

11

diplopia in ET is crossed or uncrossed?

uncrossed (stimulates nasal retinas)

12

is w4d more dissociating w lights on or off?

off

13

if the eye is turned in, on what part of the retina does the image fall?

nasal

14

pts w monofixation will fuse w4d at near or distance?

4 lights at near, 2 lights at distance

15

who gets monofixation syndrome?

post-op congenital ET who has small residual ET

16

whats the most "dissociating test" that you can use to test for arc? (most dissociative means least like an everyday binocular use of eyes

afterimage test (tests each eye individually and tags the fovea w vertical line in one eye and horizontal line in the other)

17

if you give an after image test and you see an X what does that mean

you have NRC

18

order these tests from most dissociative to least
red glass, bagolini, after image, amblyoscope, w4d

afterimage- most
w4d
red glass
amblyoscope
bagolini- least

19

How many layers are there in the LGN?

6

20

if pt has a strong fixation preference and cannot hold fixation with one of their eyes what does it mean about that eye?

it is amblyopic

21

whats the type of ambly with congenital ptosis?

aniso ambly 2/2 astigmatism

22

how many seconds of stereo is perfect stereo?

40 sec

23

how do you deternine when to operate on someone with X(T)? based on pd?

no, its all based on whether they can control the X(T) or not. Not how big it is

24

why would you give a kid over-minus glasses?

if they are XT it will cause them to accommodate more and converge more

25

whats the normal value of AC:A ratio?

5

26

what surgery do you for X(T)

LR recession OU

27

Whats the surgery for sensory XT?

R&R on amblyopic eye

28

when do you get A and V patterns?

in et or xt

29

what surgery would you do for XT w big V- IO overaction?

LR recession OU with IO weakening OR you could move the medials to the apex of the V and laterals the opposite way (MALE)

30

What chin position would a kid do if they had an A pattern?

chin down

31

how do you treat an XT with mild A pattern (ie SO overaction)

SO surgery is fraught with problems.So don't tough them, instead do MALE surgery

32

whats the formula to calculate AC:A ratio?

IPD(cm) + N-D/Diopters of accommodation
IPD= interpupillary distance
N= ET in PD at near
D= ET in PD at dist
Diopters = 3
remember to convert IPD from mm to cm

33

whats the Parks rule for determining if there is high AC:A ratio?

if their ET at near is 10PD greater than their ET at distance its accommodative eso

34

whats the treatment for acomodative eso?

glasses! give full plus

35

when do you give bifocals?

straight eyes in distance and big ET at near. So at distance they look thru plano and at near they get plus so it relaxes accomodation

36

how does plus glasses relax accommodation?

it gives you + power, so you don't have to accommodate to make your own + power

37

when do you operate on an ET and do you operate for the PD cc or sc?

operate for the deviation with glasses on. Only operate if they still have ET even with full spectacle . tell them they will need glasses even after surgery

38

whats cyclic esotropia and how do you treat?

kid who is ET one day and straight the next. Generally has a 24 cycle. Operate for the full ET, do not patch! Will cause the ET to become constant

39

whats hypermetropia?

synonym with hyperopia

40

how do you treat infantile ET?

try full + glasses first

41

what are the 3 bad complications after treatment of congenital et?

1) DVD
2) latent nystagmus
3) IO overaction

42

what rule does DVD violate?

herrings law. its like you take a saw and divide your head. The eyes don't coordinate

43

what direction is latent nystagmus?

fast phase toward the fixing eye

44

do kids with congenital ET have any chance for high grade stereopsis?

not a chance. At best they can become monofixators (small central suppression/scotoma)

45

whats smooth pursuit asymmetry?

normal kids develop smooth pusuit from temporal to nasal direction first. once they get older they can follow nasal to temporal. Kids with ambly never develop good nasal to temporal pursuit

46

how often can infantile ET resolve

almost never. only 4%

47

whats the forced generation test?

put in proparacaine, grob eye w .12 and tell them to look in each direction and feel if the eye is generating force

48

how do you treat a vascular or traumatic cn6 palsy?

observe 6 months

49

what surgery can you do for cn6 palsy?

1)hummelsheim procedure. split both the SR and IR and transpose part of it to the insertion of the LR
2) Jensen- split the LR and sew the muscle itself to the SR and IR

51

Whats double elevator palsy?

actually a monocular elevation problem
Tight IR (restrictive) vs. paralysis
Causes unable to look up in adduction and abduction (unlike browns has prob w elevation in adduction only)

51

Whats the main cause of acquired browns sx?

Sinusitis

52

How do you treat double elevator palsy?

if restrictive- recess IR
If paralytic- do Knapp procedure. Bring the MR and LR up next to the SR

53

How do you work up acquired browns?

Image the orbit

54

What does it mean if you have a hyper and the hyper is worse on tilt toward that side?

It must be an oblique problem!!!!

55

If a kid has a left hyper worse on left head tilt, what are the 2 options of which muscle is bad?

Left SO
Right IO

56

What will someone w congenital so palsy look like?

Will have head tilt and facial asymmetry able to fuse hyper

57

How do you treat congenital so palsy?

Weaken ipsilater io

58

Whats a bad complication of anyeriorizing the io to much?

Anti elevation syndrome
Bc the io elevates if you weaken too much you fuck it up

59

Why do you do a harada ito procedure?

Produces intorsion

60

whats a typical stereoacuity in monofixation syndrome?

2000 sec of arc (not good)

61

when would you operate on 1 muscle vs 2 muscles?

if the deviation is > 25PD you have to operate in 2 muscles

62

what do you do if a baby doesn't FF at 5 mo?

observe. if still not FF at 1 yr do VEP

63

whats the definition of ophthalmia neonatorum?

conjunctivitis in 1st month of life

64

oil droplet cataract associated w?

galactosemia

65

what type of cataract in alports syndrome?

ant lenticonus

66

what type of cataract in downs?

cerulean "blue dot" cataract

67

discaform cataract associated with?

lowes syndrome (x-linked recessive, MR, glaucoma)

68

how is lowe's inherited, whats the synonym, and what type of cataract

x-linked
oculocerebrorenal syndrome
discaform (50% will have cat and glaucoma)

69

for any et or xt of 15PD, how far back should your R&R be on the LR AND MR?

MR 3mm
LR 4mm

70

you do a goniotomy for a kid- it doesn't work. Whats the next step?

another goniotomy!

71

in what order do these causes of ophthalmia neonatorum present themselves? chlamydia, chemical, gonnorhea, hsv

chemical- first 24 hrs
gonorrhea- 2-3 days
chlamydia- 5-7 days
hsv- 2 weeks

72

what's megalocornea? (besides glaucoma)

x-linked condition in boys. random. moms can have enlarged K nerves

73

which antiemetic is totally contraindicated in kids <2

promethazine (resp depression- its like meth for kids)

74

which babies get screened for rop?

<1500 grams (3.3 lbs)

75

kids with myelinated rnfl are at risk for what type of amblyopia

refractive

76

Mass superior to medial canthus worry abt

Meningocele!!!!
Superior= coming from brain

77

homocystinurea- inher pattern, how do the kids look, which way does lens dislocate, complication of surgery

AR
Tall blonde with MR
inf-nasal dislocation
hypercoaguability

78

upper eyelid coloboma is associated with what syndrome?

goldenhaar

79

upper eyelid coloboma is associated with what syndrome?

goldenhaar

80

whats another name for posterior lenticonus and is it uni or bilateral?

oil droplet cataract
unilateral

81

whats a test beside an MRI that you can use to check for neuroblastoma?

urinary catecholamines

82

when do you take out
1) unilateral congenital cat
2) bilateral congenital cats

1) before 6 weeks old
2) before 10 weeks old

83

whats the primary, secondary and tertiary fxn of the SR?

1) elevation
2) intorstion
3) adduction

84

whats the inher of oculocutaneous albinism?

AR

85

Whats the difference between tyrosinase-pos and tyrosinase-neg?

tyrosinase negative has worse prognosis bc complete lack of pigment

86

whats the difference between oculocutaneous albinism and ocular albinism

ocular albinism occursonly in boys bc its X-linked recessive

87

what chromosome is associated with RB?

13

88

What chromosome is associated with alveolar rhabdo

cross between chrom 1 and 13 (13 is the rb chrom)

89

what tumor has antoni A and B cells?

Schwannoma

90

FGFR gene associated with what syndrome?

crouzons

91

how does patau syndrome (trisomy 13) present?

Microphthalmos
MR
Polydactyly
dwarfism

92

how are the Y sutures oriented in the lens?

upright Y anteriorly
inverted Y posteriorly ((posterior for peace")

93

what do you have to worry about when you put a pt w weill-marchesani under general anesthesia?

thromboembolic events

94

Most common indication for PK in kids?

peters

95

what diameter of K should make you suspicious for congenital glaucoma?

>12

96

describe the kestenbaum procedure

try to align eyes TOWARD the head turn, so if kid has left head turn, would resess LLR, RMR etc.

97

which has the worst gram + coverage?

ceftaz

98

whats the inheritance of ectopia lentis et pupillae?

AR (a closed pupil recesses you ability to see)

99

what molecule is defective in marfans?

fibrillin

100

in what dz do you give low methionine and high cysteine?

homocystinuria (AR, with MR, tall, seizures)

101

inheritance of fabrys

x-linked recessive (Fabulous HUNTER- x links the spot)

102

when do you screen rop kids?

4-6 weeks post-natal OR
31-32 wks GA

103

if you have a kid straight in dist but has accom ET- how do you treat?

give bifocal glasses
no surgery if they are straight in the distance

104

how do you treat infantile ET?

Give full +
if still have ET even with glasses then surgery

105

how much ET do you operate for in accommodative ET?

Operate on the Dist cc (The augmented way is to operate on the avg value bet the Nsc and Ncc)

106

how do you treat cyclic esotropia?

operate on the full deviation on the bad day

107

right hyper, what are the 4 possible effected muscles?

RSO
LIO
RIR
LSR

108

what does the harada ito procedure do?

strengthens fibers causing intorsion

109

Right hyper WORSE ON LEFT HEAD TILT what are the 2 possibilities?

RIR- hyper will be worse with right gaze
LSR- hyper will be worse with left gaze

110

right SO palsy what are parks 3 steps

Right hyper
Left gaze worse
Right head tilt worse

111

how does x-linked dominant work?

need only 1 X to get the dz so both boys and girls can get it

112

how does x-linked recessive work?

mom is the carrier of 1 bad X
boys get the bad X from mom and get dz
girls end up being carriers

113

what are the primary, secondary and tertiary fxns of the SO? (SO-LID)

1) intortion
2)depression
3) lateral movement of the eye

114

weill marchesani- inher and cataract type

AR
microspherophakia
short
flat head

115

galactosemia- inher, type of cataract

AR
oil-droplet (aka post lenticonus)

116

most common manifestation of fabrys in the eye

K verticilata (x linked)

117

myelinated rnfl ass with what syndrome

basal cell nevus syndrome (they dev bccs)
or ipilateral high myopia

118

how can you differentiate browns from IR restriction

there will be restriction with retropulsion in browns

119

CHED1 vs CHED 2- inheritance, how does it present?

ched 1- AD presents at 1-2 yo, progresses slowly
ched 2- AR cloudy from birth

120

congenital stroma corneal dystrophy- inher and how does it present?

AD
flaky things in stroma, doesn't get worse

121

what did the beat ROP study show?

that IVA was better than laser for stage 3 zone 1 rop

122

what chediak higashi syndrome

recurrent pyogenic infections ass with albinism

123

whats hermansky pudalk

platlet pooling problem leading to bleeding in Puerto Ricans w albinsim

124

Whats waardenberg sx?

Neural crest dz
Iris heterochromia (brilliant blue eye)
White forelock
Deafness

125

What dz ass w pax 6?

Peters
Aniridia
Coloboma
Microphthalmia

126

Skew deviation (2/2 bad vestibular input) looks like CN4 palsy in every way except

the effected eye will be INCYCLOTORTED! that is not c/w cn4 palsy

127

besides the 3 step test for cn4 palsy, what other deviation can you find w 4th?

v pattern eso (worse in downgaze!)

128

can peters be associated with glaucoma?

yes!

129

whats peters plus?

peters w systemic anomalies- facial cardiac, polydactyly

130

posterior lenticonus present at birth?

no
they are unilateral and acquired
ass w galactosemia

131

alports- inheritance, signs and retina finding

x-linked dominant
glomerulonephritis
high-tone deafness
"dot and fleck" retinopathy

132

in latent nystagmus when you occlude one eye what happens to the velocity of the nystagmus

DECREASE in slow phase velocity

133

in congenital nystagmus what happens to the velocities over time?

INCREASE in the slow phase velocity

134

whats the definition of megalocornea?

>13mm in diameter

135

what dz can be associated with megalocornea?

marfans

136

kids w forecep injury lose vision from what

astigmatic amblyopia (the k edema from the forceps resolves quickly so its not the cause of the ambly)

137

whats the general surgical approach to duanes?

RECESS the muscle that's actually working in the effected eye.

138

2 diseases associated w Christmas tree cataract

myotonic dystrophy
hypoparathyroidism (low ca levels)