2. DUANE’S SYNDROME
aka Duane’s retraction syndrome
a congenital eye movement disorder due to misdirection
of the nerve fibre on eye muscle
causing some eye muscle to contract when they shouldn’t, vice
versa
can be unilateral or bilateral
3. Aetiology:
1.
Mechanical factors
fibrosed LR
abnormally insertion of MR
binding of MR sheath to wall
2.
Embryonic factors
Disturbance in normal embryonic development during 2nd
month of gestation
3.
4.
Development of 3rd, 4th, and 6th cranial nerve occur
Paradoxical innervation
Increase innervation to both MR & LR during ADD and
relaxation of both MR & LR during ABD
Trauma
4. Clinical features:
limitation of abduction with or without limitation of
adduction
attempt adduction: retraction of the globe with
narrowing of fissure
Protrusion & widening of the palpebral fissure on
abduction
May or may not have AHP
Strabismus
Amblyopia
5. PATIENT: MALE/10/MALAY
FILE NO: 0377
Date: 28th February 2012
CHIEF COMPLAINT
-complaint of blur when seeing distance, want to make spectacles
-has problem in writing ; words become shuffle since kindergarten
OCULAR HISTORY
-Father claimed has lazy eye since 2-3 years old, has follow up at Hosp.
Terengganu
-easily fall when young, suspect due to lazy eye
-wear spect at very young age but refused to wear till now
GENERAL
-Asthma, eczema, use homeopathy
FAMILY HISTORY
-Mother has DM, HPT since 8mo ago
6. RE
VA
*with marked
AHP
: face turn R
LE
6/12 PH: 6/9+
N5 @ 15cm
PRIMARY
GAZE
6/24
Not able to read Snellen on
primary gaze
AHP
D
N
moderate XP
PRIMARY
GAZE
COVER
TEST
6/24 PH: 6/18+2
N5 @ 15cm
D
N
large L XT
HIRSCHBERG
central
Reflex at nasal
AA
13,13,13
13,13,13
NPC
11cm LE deviates out
STREOPSIS
240 arc (TNO)
*not reliable
7.
8. 1.
LE Exotropia on primary gaze
2.
V pattern
LE has no problem when attempt on
abduction (towards temporal)
3.
Abduction on elevation: normal
Abduction on depression: normal
LE has limitation on adduction
(towards nasal)
4.
Down shooting of LE on adduction
Also limitation of adduction on elevation
& adduction on depression
LE presents narrow palpebral fissure,
and globe retraction on adduction
11. DIAGNOSIS
1. Low myope moderate astig
2. L Duane’s syndrome
MANAGEMENT
1. Prescribe Rx
2. Write referral letter to Hosp. Terengganu
3. Refer pt to BV clinic for further assessment - 9 Apr 2012
4. KIV AA with Rx
12. The major complaint that pt has is blur at distance
More specific- since when? is it sudden or gradually
blur?
Father claimed has lazy eye since young, should wear
glasses but refuse
could be related to chief complaint : blur- possible
uncorrected refractive error
The reason of failure wear glasses?
Ask more about symptoms of amblyopia. Any eye turning in?
Eye rubbing? See at very close distance?
13. DISCUSSION: CLINICAL FINDINGS
Reduction in vision BE
1.
Improvement with pinhole: reduction of vision can be
corrected with spectacles
However vision is taken with marked AHP
Visual acuity with head straight shows further decrement in
vision on LE
Marked AHP- face turn R
2.
Visual acuity is better on LE
however marked large exo-deviation on LE in primary
position
face turn R (non deviating side) is significant as to obtain
fusion.
14. AHP
• Left exotropia. As face turn to right (non affected side), it
will compensate the deviation.
• To improve visual acuity
• To decrease deviation, hence strengthen BSV. Cover test shows
moderate XP with AHP compared to primary gaze
PRIMARY GAZE
FACE TURN
RIGHT
FACE TURN RIGHT
15. Refractive error
4.
Unaided VA RE 6/24 is correlate with refraction finding
-0.50/-3.00DCx10 (estimated astigmatism ~±3.00DC).
Unaided VA LE 6/12 is correlate with refraction finding
-0.50/-1.75X170 (estimated astigmatism ~±1.25DC).
Most astigmatism power comes from corneal astigmatism.
BE develop meridional amblyopia (6/9 BE) due to
uncorrected astigmatism.
16. Diagnosis 1: low myope with moderate astig
1.
Uncorrected moderate amount of astig presenting with
reduce of vision
Should suggest meridional amblyopia?
Management: prescribe Rx to provide optimum correction
& prevent moderate amblyopia
Diagnosis 2: Left Duane’s syndrome
2.
Limitation of adduction on LE
On attempt of adduction, affected eye appear smaller
(palpebral fissure narrowing, globe retraction), and down
shoot. Classic sign on Duane’s Type 2.
There is 4 types of Duane’s syndrome
Management: refer to Hospital and BV clinic
17. Refer to BV: patient came on 2nd April 2012
Additional test- Hess chart: to investigate incomitant
strabismus in order to asses paretic element
LE
1. LE has smaller field than RE. Suggest LE affected eye.
2. Sloping sides to field indicates V pattern.
3. Compressed field of LE on nasal part.
• Underaction of Left MR, IO, SO
4. Larger field of RE
• Overaction of Right SR, LR, IR
RE
18. TYPE 1
TYPE 2
TYPE 3
TYPE 4
-Poor abduction,
good adduction
-Poor adduction,
good abduction
-Poor adduction,
poor abduction
-Paradoxical
abduction on
attempt adduction
-agenisis of 6th nerve
-3rd nerve split
innervate LR, MR
-adduction intact as
most nerve goes to
MR
-6th nerve intact
-3rd nerve split
innervate LR, MR
-Poor adduction as
LR contract against
MR
-6th nerve agenesis
-3rd nerve split
innervate LR, MR
-The split is equal
-Eye not moves
in/out
-6th nerve agenesis
-3rd nerve split
innervate LR, MR
-most innervate LR
-when ADD it ABD
19. TYPE 1
(70-80%)
ie: LE
LE
Esotropia
with head
straight
Face turn to
affected
side
TYPE 2
(~7%)
ie: LE
LE
Exotropia
with head
straight
TYPE 3
(~15%)
TYPE 4
ie: LE
Eyes are
aligned in
primary
position with
head straight
ie: LE
Large LE
Exotropia
Face turn to
nonaffected
side
Limited
abduction
left eye
Normal or
less
abduction
-Normal
or less
adduction
-Narrowing
of fissure
-Globe
retraction
-Limited
adduction
-Narrowing
of fissure
-Globe
retraction
-Marked upshoot and
sometimes downshoot
on adduction
Limited
abduction
left eye
-Limited adduction
-Narrowing of fissure
-Globe retraction
-Upshoot/ down shoot
Limited
adduction
RE
Simultaneous
abduction when
looking toward
uninvolved side
-violating Hering’s law
20. Management of Duane’s syndrome
Correct refractive error
Treat amblyopia
In this case, no patching treatment is indicated yet as vision BE is
almost similar
Meridional amblyopia usually has good prognosis with spectacles
alone
To monitor vision after correction after 3 months.
Surgery indicated if:
Marked AHP
Decompensating
Cosmetically poor deviation
Diplopia occurring more frequently
21. Refer to ophthalmology
To perform additional test for further evaluation
Forced duction test: to evaluate muscle palsy (+ve forced
duction test)
Suggestion for squint surgery
The marked AHP is consistent
To improve cosmesis & comfort to patient
22. Duane’s syndrome is a congenital eye movement disorder in
which there is miswiring of the eye muscles that typically
can be recognized through a few ocular signs and
symptoms.
As an optometrist, we should smartly recognized this
syndrome according to the history taking and clinical
findings in order to make an accurate diagnosis.
Although the syndrome is permanent, further managements
is crucial in order to solve patient’s problems such as marked
AHP and also on.
23. 1. Fiona J. Rowe. Clinical orthoptics. 3rd edition.
2.
3.
4.
5.
Wiley-blackwell.
http://emedicine.medscape.com/article/119855
9-overview date: 20th April 2012
http://telemedicine.orbis.org/bins/volume_page.
asp?cid=1-3-5-50
date: 20th April 2012
http://www.webmd.com/eye-health/duanesyndrome
date: 20th April 2012
http://childrenshospital.org/az/Site3103/mainpa
geS3103P0.html
date: 20th April 2012
Duane syndrome: A congenital eye movement disorder in which there is miswiring of the eye muscles, causing some eye muscles to contract when they should not and other eye muscles not to contract when they should. People with the syndrome have a limited (and sometimes no) ability to move the eye outward toward the ear (to abduct the eye) and, in most cases, a limited ability to move the eye inward toward the nose (to adduct the eye). Often, when the eye moves toward the nose, the eyeball also pulls into the socket (retracts), the eye opening narrows and, in some cases, the eye moves upward or downward. Many patients with Duane syndrome turn their face to maintain binocular vision and compensate for improper turning of the eyes.
Bluring of vision at distance suggest uncorrected refractive errorHas significant ocular history regarding Should ask more the condition of blur vision. -Is it gradually decrease/ becoming worse or persistent blur vision?-
Reduction in vision BE-Improvement with pinhole: part of reduction of vision can be corrected with spectacles-However vision is taken with marked AHP-Visual acuity with head straight shows further decrement in vision on LE2. Marked AHP- face turn R -vision is better on LE -however marked large exo-deviation on LE. AHP: face turn R (non deviating side) is significant as to obtain fusion. Large L XT is correlate with corneal reflex displaced nasally and AHP: face turn to right.
MRunderaction -4SO overaction +2
2. Marked AHP- face turn R -vision is better on LE -however marked large exo-deviation on LE. AHP: face turn R (non deviating side) is significant as to obtain fusion.