Transcript Special Forms of Strabismus Types of strabismus that often break

A and V Patterns and
Other Types of
Strabismus
Paviglianiti / January 21, 2010
A and V Patterns








Horizontal deviations that change in magnitude in upgaze and
downgaze
A or V patterns reportedly in 15-25% of all horizontal strabismus
IOOA assoc with V patterns
SOOA assoc with A patterns
Sometimes, if vertical muscles underacting (eg SR) then their tertiary
adducting effect in upgaze will decrease, giving a V pattern;
underacting IR will decrease its tertiary adduction in downgaze, giving
an A pattern (both SR and IR are tertiary adductors)
Pts with Aperts/Crouzons have V patterns since their orbits are
excyclotorted
Technically, need a difference between upgaze vs downgaze of at least
10d in A pattern; 15d in V pattern (I use 15 for both)
If obliques are overacting, weaken them; if not, do MALE horizontal
transposition by ½ tendon width…rules apply regardless of whether you
are weakening or tightening or whether surgery on 2 eyes or R/R on 1.
Special Forms of Strabismus

Duanes Syndrome
 Möbius Syndrome
 Brown’s Syndrome
 Thyroid Eye Disease
 Congenital Fibrosis Syndrome
 Progressive External Ophthalmoplegia (PEO)
 Myasthenia
 Internuclear Ophthalmoplegia (INO)
 Congenital Oculomotor Apraxia
 Congenital / Acquired 3rd, 4th, 6th palsies
Concomitant vs Incomitant
 Concomitant
Strabismus:
 The
measurements are the same in all
fields of gaze (numbers on the tictactoe
board are the same in all the boxes) AND
the range of mvt of the eyes is full
 Incomitant
Strabismus
 Measurements
gaze
different in different fields of
Duane’s Syndrome
 Type
I =>reduced abduction
 Type II => reduced adduction
 Type III => reduced abd and add
 Clinically,
little use
this classification scheme is of
Duane’s Syndrome/Type I
Can’t abduct, so usually esotropic in primary
position, sometimes with compensatory head
turn for fusion
 Usually girls
 More often left eye, but bilateral 20%
 On attempted abduction, involved lids widen



Involved lids widen on attempted mvt toward
deficit
On adduction, involved lids narrow and globe
retracts into orbit, and there are upshoots and
downshoots

Involved lids narrow on attempted mvt away from
deficit; upshoots/downshoots on involved eye with
attempted mvt away from deficit
Bilateral Duane’s I
Bilateral Duane’s; note
lid widening on
attempted abduction
 Note the working
adducting eye with lids
narrowing and globe
retraction

Duane’s Syndrome
Type II
 Limited
adduction, so XT in primary
 Ddx includes INO, but INO has no lid
fissure widening on attempted
movement toward the deficit
Duane’s II


Note full abduction,
limited adduction with
upshoot on attempted
right gaze
Could confuse with INO
or IOOA
Duane’s Syndrome
Type III
 Limited
abduction and adduction, so
usually ortho in primary
Duane’s Syndrome

Usually NOT inherited (sporadic), but 10%
there is auto dominant inheritance
 Since bilateral 20%, ddx includes congenital
esotropia with tight medial recti, which don’t
allow full abduction
 Used to be thought it was caused by
hypoplasia of 6th nerve nucleus, with the 3rd
nerve taking over innervation of the LR in an
abnormal way; this isn’t proven and exact
cause of Duane’s still mysterious
 Ocular associations: ptosis of involved side;
cataracts, iris heterochromia, iris/retina
coloboma
Duane’s Syndrome
Associations

Thalidomide exposure (West Germany 19571962: 30% of these babies had Duane’s
 Possible fetal alcohol link (so teratogenicity
during 4th to 5th weeks of gestation possible?)
 Deafness in 10% of Duane’s (although I
haven’t noted that clinically)
 Duane’s + deafness + Klippel/Feil spine
anomaly (short neck, reduced mvts, low
posterior hairline) => WILDERVANKS
(cervico-oculo-acoustic)
 Goldenhar’s
 Marcus-Gunn jaw winking and crocodile tears
(other forms of aberrant innervation)
Duane’s / Management
Ability to abduct “will never get better”
 It is NOT possible to create “normal” eye mvts
via surgery
 Treat any underlying amblyopia;
 Check for hearing deficits if indicated
 Indications for surgery: head turns (to try to
gain ortho and fusion) or big tropia in primary
 Resist the urge to resect (tighten) the affected
muscle: will increase retraction and the
muscle doesn’t work well anyway. Some
have even proposed weakening the affected
LR more to decrease retraction
 Usually begin with MR recession; can do
transpositions of SR and IR to LR with
concurrent MR recession

Möbius Syndrome

6th and 7th nerve palsies BUT…


Motility is more than just limited abduction; may be
some limited adduction as well (sometimes partial
3rd)as well as some elevation defects
Mask-like facies may be incomplete (may spare
lower face

Check for incomplete lid closure, corneal
anesthesia, and exposure
 Accompanied by limb, chest, tongue
problems



Agenesis/dysgenesis of limbs; terminal limb
problems like polydactyly or syndactyly
Check for absent pectoralis muscle (Poland’s
Anomaly)
Tongue hypoplastic; dental malocclusion
Möbius Syndrome

Postmortem evidence shows cause is
agenesis of 6th, 7th, and 12th cranial nerve
nuclei (the EOMs themselves are
normal…though may become fibrotic from
lack of innervation)
 Ophtho Management



medial rectus recession for abduction deficits
Lubricate corneas if not good lid closure; lateral
tarsorrhaphy to protect corneas if needed
Referral for speech eval, cardiac eval, and
endocrine eval (can have heart and
endocrine problems too)
 Usually only 10% mentally delayed
 Most are rejected and depressed due to facial
appearance
Möbius Syndrome

Brown’s Syndrome
Unable to elevate the adducted eye

Restricted on forced duction testing

Cause: developmental anomaly of the SO
tendon (the trochlea and the SO tendon
derive from the same mesenchymal tissue; in
utero, these get tangled up and cause
tethering / restriction of SO tendon
 Usually congenital, but can be acquired


DDx: IO underaction and SO overaction


Trauma or surgery to canthal area, inflammation,
or metastasis to area
These do not have positive forced ductions
10% are bilateral
 Sometimes runs in families (always bilateral)
Brown’s Syndrome

Kids present with abnormal head posture, or parents
saying “eyes just don’t move right when pt looks up at
me”
 Usually will note EXOtropia in upgaze
 Treat for significant head posture or significant
hypotropia / diplopia in primary, loss of stereo
 Options: none good: SO weakening via SO
tenectomy (but can over-do, and cause SO paresis);
silicone expander (fallen out of favor); most need at
least 2 re-ops
 Leave these kids alone unless forced to do
something: reported tendency toward improvement
as child get older, as abnormal connections between
the trochlea and SO break down; but not always:
some adults still have it;
Brown’s Syndrome
Thyroid Eye Disease

Restrictive myopathy with lymphocytic
infiltration of EOMs
 Usual order for boards IMSLow (IR affected
most>MR>SR>LR; from recent lecture by Dr.
Gearity, the 3 muscles are affected about the
same.
 Usual misalignment is proptosis, hypotropia,
and esotropia (from scarred/fibrosed IR
pulling eye down; fibrosed MR pulling eye in);
forced ductions are usually positive
 Present with diplopia that does not follow any
pattern, therefore prisms usually don’t work
Thyroid Eye Disease

Most are hyperthyroid at dx, but can be
euthyroid or hypothyroid
 Get TSI, TSH, and T4 (per Dr. Gearity)
 CT orbits can be helpful to see degree of
EOM involvement
 Thyroid eye disease often co-exists with
myasthenia so eval for this also
 If optic nerve involved, may need orbital
decompression prior to strabismus surgery
 Surgery: optional oral prednisone for 2 weeks
(“to soften things up”); then surgery (ie
inferior rectus recession: aim to undercorrect
Thyroid Eye Disease
Congenital Fibrosis
Syndromes

Group of congenital anomalies with variable
amts of restriction of EOMs via replacement
of muscle tissue with fibrous tissue
 Can be single muscle on one eye to all
muscles on both eyes
 Often levator involved=>ptosis=>chin up
head posture
 DDx is PEO (frozen eyes and ptosis)
 Many types inherited (auto dominant usually)
though can be sporadic
 Etiology unknown
 Goal: get ortho in primary
Congenital Fibrosis
Syndromes

General fibrosis of all the EOMs (mostly auto
dominant inheritance)
 Congenital fibrosis of the IR=>cannot look up;
can mimic double elevator palsy
 Congenital fibrosis of the MR/LR (strabismus
fixus)


Can present with congenital esotropia appearance
with both eyes pointing down and in toward nose
Congenital fibrosis of the SR=>pt cannot look
down
Congenital Fibrosis - familial
Chronic Progressive External
Ophthalmoplegia (CPEO)

Presents as ptosis, followed by progressive
paresis of EOMs; pupils spared
 DDX is Congenital Fibrosis (frozen eye and
ptosis)
 Triad of PEO, pigmentary retina changes and
heart block/cardio problems is Kearns-Sayre,
(usually before age 20) (many times a board
question); mgmt: refer for sequential EKG



Other signs of K-S: short stature, hearing loss,
diabetes/endocrine problems
Dx: EOM or limb muscle bx show “ragged red
fibers” (actually are clumps of degenerated
mitochondria) on trichrome stain
May be mitochondrial dysfunction as cause
Myasthenia
 Look
for variable strabismus throughout
the day or on sequential exams
 Diplopia is often intermittent
 Can be “ocular “ only or systemic
 Look for fatigueability on extended
upgaze
 Often reversed by a few minutes of cold
ice in a rubber glove on the eyelids
Myasthenia

Refer to Neuro for Tensilon test / EMG




Test dose 0.1cc edrophonium (tensilon)
If ok, then 0.9cc edrophonium
Keep IV atropine on hand as antidote
Can test for

Acetylcholine receptor antibody


Antistriated muscle antibody


Found in 90% of acquired MG (not found in congenital
MG); only present 50% of time in “ocular” MG
Found in 90% of MG with thymoma and 30% of MG pts
without thymoma
Therefore, in typical diplopia pt with
fatigueability, but no systemic sx, these tests
will be negative often despite disease, so still
refer to neuro for tensilon test if suspicious
Internuclear Ophthalmoplegia
(INO)
Looks like a “one-sided MR palsy” (ipsilateral
to the lesion), with the other eye having
horizontal nystagmus
 The limited adduction may be total or partial
 Both eyes adduct normally on convergence to
a near target
 Is a problem in the MLF (medial longitudinal
fasciculus) of the brain stem
 Interruption in signal from from abducens
relay signals from reaching the contralateral
oculomotor nucleus, impairing adduction

INO
Anatomy
 IF
I WANT TO LOOK LEFT:
 Left PPRF stimulates the Left 6th N
nucleus=> (abduct left eye)
 Left 6th N nucleus stimulates the MLF
 MLF crosses and ascends to the
contralateral (right) 3rd nucleus
 To give contralateral (right) MR
contraction (adduct right eye)
INO
 In
adults: Multiple Sclerosis is common
cause and many times is bilateral INO
 In kids: posterior fossa tumors (eg
brainstem glioma), Arnold-Chiari, head
trauma, sickle cell
 In old folks: frontal lobe CVA or tumor
One and a Half Syndrome
(INO + )(Fisher’s Syndrome)

Injury to the PPRF (also known as the lateral
gaze center) or the 6th nerve nucleus causes
loss of ipsi abduction and contralateral
adduction) (the one)
 Injury to the ipsi MLF (knocks off ipsi
adduction) (the half)
 The only mvt remaining is the contralateral
abduction (via the contralateral 6th N nucleus
 DDx: congenital fibrosis (forced ductions give
the answer)
Congenital Oculomotor Apraxia
(Saccade Initiation Failure)
 These
folks cannot voluntarily initiate
horizontal eye mvts => see head
thrusting to look to sides
 Vertical mvts intact
 Get MRI to look for head tumors
 If acquired oculomotor apraxia in adult,
look for parietal CVA, mets, or trauma
 Etiology unknown
3rd, 4th, and 6th Nerve Palsies
Congenital/Acquired
 Survey
of 160 palsies in kids
were 6th N palsies
 25% were 3rd N palsies
 25% were 4th or multiple N palsies
 50+%
 Trauma
most common cause in kids
rd
3

Nerve Palsies
Congenital




Rare
Look for ptosis and exotropia (with mixture of strab
due to reduced adduction, elevation, and
depression) and pupil involvement (the eye is
down and out due to unopposed LR and SO (a
depressor)
Pupil usually dilated
Look for aberrant regeneration due to misdirection
of any 3rd N fibers that actually are working
rd
3

N Palsies
Acquired (kids)





Trauma (most common cause of acquired 3rd N
Post-viral
Infectious (13/147 cases of meningitis had 3rd N
palsies; Hflu, Nmeningitidis, Spneumoniae)
Inflammatory
Migraine (ophthalmoplegic migraine)





Pain around the eye for 24-48 hrs, with N/V, then as pain
lessens, a 3rd N palsy develops, can last for a few days
to a few months
Recurrent, usually a family h/o migraine
First attack usually before age 12
More common in boys than girls
Tumor (eg brainstem glioma)
rd
3
 Acquired
N Palsies
(adults)
 Aneurysm
(youngest age for PCOM
aneurysm is 14 so far); “angiography to
exclude an aneurysm is NOT justified
below the age of 10” (Taylors text)
 Diabetes (usually resolves over 6 months)
 Trauma
 Infection
 Tumor
rd
3

Nerve Palsy
Ptosis, exotropia and
hypotropia (only the LR
and SO working…so
eye is down and out)
and blown pupil
4th Nerve Palsy

Congenital



Look for the head tilt; do measurements with head
straight, to show the strabismus
Ddx of head tilt, with hypertropia in primary gaze is
IOOA, DVD, Duane’s with upshoot
Bielschowsky: LRL: means LHT, worse on right
gaze, worse on L head tilt (is L SO4 palsy) (not to be
confused with LLL which is RIO palsy)




Look for excyclotorsion
Look for facial asymmetry (hypodevelopment/
thinner face on contralateral side)
Many times these present later in life; looking at
old photos shows the head tilt present in childhood
and they have large fusional amplitudes
Causes: frequently birth trauma or just bad
anatomy (weak SO tendon)
th
4

Acquired


Note: since the 4th N has the longest course and is
the only one to exit on the dorsal surface of the
brain stem, so it the most vulnerable to damage
from trauma
Causes:







N Palsy
Trauma (#1)
Diabetes / ischemia (#2)
Hydrocephalus / Tumors
Post infectious
Demyelinating disease
Commonly: No cause found
Surgical Mgmt of SO4 palsy: IO myectomy +/SO tuck +/- additional vertical rectus surgery
Oblique Palsies and
Overactions - TORSION


1st photo shows normal relationship of fovea to disk with respect
to torsion. Normal fovea location is between two imaginary
lines drawn thru middle of disk and lower margin of disk (direct
view)
Inferior oblique overaction / SO palsy could show the right
fundus above; [note that on your 20Diopter INDIRECT view, the
fovea should be at upper half of disk, and torsion will make
fovea seem way up, as you view it with indirect]
th
6
Nerve Palsy
 Congenital
 Duane’s
 Transient
LR palsy of Newborns, usually
goes away before it is even diagnosed
(may be as common as 1/ 182 normal
births); sometimes there is h/o birth trauma
or forceps
th
6

N Palsy
Acquired





Trauma (40%)
Neoplasms (16%) (pontine glioma)
Intracranial Hypertension (Pseudotumor or
hydrocephalus)
Infection (meningitis, varicella, Lyme) and s/p
vaccination (MMR)
Acquired 6th N palsy of unknown cause in children:
isolated, painless 6th N palsy that resolves in 8-12
weeks; w/u negative (including MRI and CSF and
neuro w/u); may recur
th
6

Nerve Palsy
6th Nerve palsy in a 10yo male who had a
brainstem glioma
rd
th
thv1v2
th
3 /4 /5
/6
 Image
combo palsy
the cavernous sinus area
 If there is proptosis, consider an orbital
apex syndrome