Flashcards in Peds Deck (139):
What cells is neuroblastoma derived from?
Precursor cells for the sympathetic pathway. Therefore it excretes catacholamines
What are racoon eyes a sign of?
Congenital horners should raise suspicion for
Neuroblastoma bc it messes w the sympathetic pathway
Check urine catacholamines
What do eyes look like in primary position in browns syndrome?
hypotropia of effected eye
Endothel dystrophy (ched 2 AR cloudy from birth)
on eom in browns what do you see
unable to elevate when adducted improves upon abduction
how do you treat duanes surgically?
recess the (tight) medial rectus of effected eye
how do you surgicaly treat upshoots and downshoots in duanes?
Y split of lateral rectus of effected muscle
objects that fall on the horopter stimulate what?
corresponding retinal points
whats kassebach-merrit syndrome
in capillary hemangioma there may be plt sequestration causing thrombocytopenia. so check cbc in kid w cap hemangioma
diplopia in XT is crossed or uncrossed?
crossed (stimulates temporal retinas)
diplopia in ET is crossed or uncrossed?
uncrossed (stimulates nasal retinas)
is w4d more dissociating w lights on or off?
if the eye is turned in, on what part of the retina does the image fall?
pts w monofixation will fuse w4d at near or distance?
4 lights at near, 2 lights at distance
who gets monofixation syndrome?
post-op congenital ET who has small residual ET
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)
if you give an after image test and you see an X what does that mean
you have NRC
order these tests from most dissociative to least
red glass, bagolini, after image, amblyoscope, w4d
How many layers are there in the LGN?
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
whats the type of ambly with congenital ptosis?
aniso ambly 2/2 astigmatism
how many seconds of stereo is perfect stereo?
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
why would you give a kid over-minus glasses?
if they are XT it will cause them to accommodate more and converge more
whats the normal value of AC:A ratio?
what surgery do you for X(T)
LR recession OU
Whats the surgery for sensory XT?
R&R on amblyopic eye
when do you get A and V patterns?
in et or xt
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)
What chin position would a kid do if they had an A pattern?
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
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
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
whats the treatment for acomodative eso?
glasses! give full plus
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
how does plus glasses relax accommodation?
it gives you + power, so you don't have to accommodate to make your own + power
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
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
synonym with hyperopia
how do you treat infantile ET?
try full + glasses first
what are the 3 bad complications after treatment of congenital et?
2) latent nystagmus
3) IO overaction
what rule does DVD violate?
herrings law. its like you take a saw and divide your head. The eyes don't coordinate
what direction is latent nystagmus?
fast phase toward the fixing eye
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)
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
how often can infantile ET resolve
almost never. only 4%
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
how do you treat a vascular or traumatic cn6 palsy?
observe 6 months
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
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)
Whats the main cause of acquired browns sx?
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
How do you work up acquired browns?
Image the orbit
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!!!!
If a kid has a left hyper worse on left head tilt, what are the 2 options of which muscle is bad?
What will someone w congenital so palsy look like?
Will have head tilt and facial asymmetry able to fuse hyper
How do you treat congenital so palsy?
Weaken ipsilater io
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
Why do you do a harada ito procedure?
whats a typical stereoacuity in monofixation syndrome?
2000 sec of arc (not good)
when would you operate on 1 muscle vs 2 muscles?
if the deviation is > 25PD you have to operate in 2 muscles
what do you do if a baby doesn't FF at 5 mo?
observe. if still not FF at 1 yr do VEP
whats the definition of ophthalmia neonatorum?
conjunctivitis in 1st month of life
oil droplet cataract associated w?
what type of cataract in alports syndrome?
what type of cataract in downs?
cerulean "blue dot" cataract
discaform cataract associated with?
lowes syndrome (x-linked recessive, MR, glaucoma)
how is lowe's inherited, whats the synonym, and what type of cataract
discaform (50% will have cat and glaucoma)
for any et or xt of 15PD, how far back should your R&R be on the LR AND MR?
you do a goniotomy for a kid- it doesn't work. Whats the next step?
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
what's megalocornea? (besides glaucoma)
x-linked condition in boys. random. moms can have enlarged K nerves
which antiemetic is totally contraindicated in kids <2
promethazine (resp depression- its like meth for kids)
which babies get screened for rop?
<1500 grams (3.3 lbs)
kids with myelinated rnfl are at risk for what type of amblyopia
Mass superior to medial canthus worry abt
Superior= coming from brain
homocystinurea- inher pattern, how do the kids look, which way does lens dislocate, complication of surgery
Tall blonde with MR
upper eyelid coloboma is associated with what syndrome?
upper eyelid coloboma is associated with what syndrome?
whats another name for posterior lenticonus and is it uni or bilateral?
oil droplet cataract
whats a test beside an MRI that you can use to check for neuroblastoma?
when do you take out
1) unilateral congenital cat
2) bilateral congenital cats
1) before 6 weeks old
2) before 10 weeks old
whats the primary, secondary and tertiary fxn of the SR?
whats the inher of oculocutaneous albinism?
Whats the difference between tyrosinase-pos and tyrosinase-neg?
tyrosinase negative has worse prognosis bc complete lack of pigment
whats the difference between oculocutaneous albinism and ocular albinism
ocular albinism occursonly in boys bc its X-linked recessive
what chromosome is associated with RB?
What chromosome is associated with alveolar rhabdo
cross between chrom 1 and 13 (13 is the rb chrom)
what tumor has antoni A and B cells?
FGFR gene associated with what syndrome?
how does patau syndrome (trisomy 13) present?
how are the Y sutures oriented in the lens?
upright Y anteriorly
inverted Y posteriorly ((posterior for peace")
what do you have to worry about when you put a pt w weill-marchesani under general anesthesia?
Most common indication for PK in kids?
what diameter of K should make you suspicious for congenital glaucoma?
describe the kestenbaum procedure
try to align eyes TOWARD the head turn, so if kid has left head turn, would resess LLR, RMR etc.
which has the worst gram + coverage?
whats the inheritance of ectopia lentis et pupillae?
AR (a closed pupil recesses you ability to see)
what molecule is defective in marfans?
in what dz do you give low methionine and high cysteine?
homocystinuria (AR, with MR, tall, seizures)
inheritance of fabrys
x-linked recessive (Fabulous HUNTER- x links the spot)
when do you screen rop kids?
4-6 weeks post-natal OR
31-32 wks GA
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
how do you treat infantile ET?
Give full +
if still have ET even with glasses then surgery
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)
how do you treat cyclic esotropia?
operate on the full deviation on the bad day
right hyper, what are the 4 possible effected muscles?
what does the harada ito procedure do?
strengthens fibers causing intorsion
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
right SO palsy what are parks 3 steps
Left gaze worse
Right head tilt worse
how does x-linked dominant work?
need only 1 X to get the dz so both boys and girls can get it
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
what are the primary, secondary and tertiary fxns of the SO? (SO-LID)
3) lateral movement of the eye
weill marchesani- inher and cataract type
galactosemia- inher, type of cataract
oil-droplet (aka post lenticonus)
most common manifestation of fabrys in the eye
K verticilata (x linked)
myelinated rnfl ass with what syndrome
basal cell nevus syndrome (they dev bccs)
or ipilateral high myopia
how can you differentiate browns from IR restriction
there will be restriction with retropulsion in browns
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
congenital stroma corneal dystrophy- inher and how does it present?
flaky things in stroma, doesn't get worse
what did the beat ROP study show?
that IVA was better than laser for stage 3 zone 1 rop
what chediak higashi syndrome
recurrent pyogenic infections ass with albinism
whats hermansky pudalk
platlet pooling problem leading to bleeding in Puerto Ricans w albinsim
Whats waardenberg sx?
Neural crest dz
Iris heterochromia (brilliant blue eye)
What dz ass w pax 6?
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
besides the 3 step test for cn4 palsy, what other deviation can you find w 4th?
v pattern eso (worse in downgaze!)
can peters be associated with glaucoma?
whats peters plus?
peters w systemic anomalies- facial cardiac, polydactyly
posterior lenticonus present at birth?
they are unilateral and acquired
ass w galactosemia
alports- inheritance, signs and retina finding
"dot and fleck" retinopathy
in latent nystagmus when you occlude one eye what happens to the velocity of the nystagmus
DECREASE in slow phase velocity
in congenital nystagmus what happens to the velocities over time?
INCREASE in the slow phase velocity
whats the definition of megalocornea?
>13mm in diameter
what dz can be associated with megalocornea?
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)
whats the general surgical approach to duanes?
RECESS the muscle that's actually working in the effected eye.