I have another case presentation during attachment at Hospital Sultanah Bahiyah, Alor Setar.

1. Prepared by:
Anis Suzanna Binti Mohamad
Optometrist
Hospital Langkawi

2. Introduction
Aetiologies
Clinical features
Classifications
Case study
Discussion
Conclusion
References

3. Binocular
vision
Non
strabismus
Strabismus
Incomitant
Comitant
Neurogenic
palsy
Myogenic palsy
Mechanical
palsy
Duane’s
Syndrome
Vertical
Retraction
Syndrome
Brown’s
Syndrome
Marcus Gunn
Jaw Winking
Orbital Injuries

4.  aka
Duane’s retraction syndrome or Stilting-Turk
Syndrome
 a congenital eye movement disorder due to
misdirection of the nerve fiber on eye muscle

causing some eye muscle to contract when they
shouldn’t, vice versa
 can
be unilateral or bilateral
 Usually congenital but may be acquired
 Affects females to males in ratio of 3:2
 May be associated with other defects such as
deafness, anisometropia etc.

5. 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

6. 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
 Possible defect of convergence
 Strabismus
 May have updrift or downdrift on adduction with A or V
pattern
 Amblyopia if not adopted AHP
 Generally positive forced duction test (done by
ophthalmologist)


7.  Clinically,
it is often subdivided into 4 types with
associated symptoms:




1. Type 1
2. Type 2
3. Type 3
4. Type 4
 Each
of the group can be further classified into 3
subgroups depends on where the eyes are when on
the primary gaze:



Subgroup A (Effected eye looks esotropia)
Subgroup B (Effected eye looks exotropia)
Subgroup C (Effected eye looks almost orthophoria)

8. 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
-agenesis 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

9. 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
non-affected
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

10. PATIENT: MALE/8/MALAY
ID NO: AS00022278
Date: 1st February 2012 (GEN clinic)
CHIEF COMPLAINT
-Referral from optometrist Kulim Hospital.
OCULAR HISTORY
-LE Duane’s retraction syndrome type A.
- Incommittant angle exophoria at primary gaze.
- No diplopia.
GENERAL
-Under peads SOPD follow-up for omphalocele at birth and bilateral undescended
testis.
- Curently patient in primary school and sit in second row in class. Able to read
blackboard without glasses.
FAMILY HISTORY
-Unremakable

11. CLINICAL FINDINGS
RE
LE
VA ( unaided)
6/24, Pinhole: 6/12-2
6/12, Pinhole: 6/9
Refraction (done
in Kulim Hospital
Pl/-1.00x10 (6/9)
Pl/-1.00x175(6/9+2)
 Diagnosis:


BE astigmatism.
LE Duane’s retraction Syndrome Type A.
 Management


and plan:
Cyclo RA appointment and review by General Follow-up
Clinic on 1/12.
KIV prescribed if needed.

12. FIRST VISIT
Date: 23rd April 2012 (OPTOM clinic)
CHIEF COMPLAINT
-Father claimed that patient not aware of vehicles
while cycling.
- Father claimed that sometimes when patient want to
focus on something, he will chin down.
OCULAR HISTORY
- Never wear glasses before.

13. RE
LE
6/12, N5
6/7.5, N5
K-reading
38.25@11
40.00@101
38.50@5
36.50@95
Refraction
+0.25/-1.75x10
(6/6-1)
Pl/-1.25x180
(6/6)
VA (unaided)
AHP
Sometimes chin down
Hirschberg
Intermittent LE small XT < degree.
Cover test
(33cm): Small XP with fast recovery
(6m): Small XP with fast recovery
OMT
SR u/a -1
NPC
LR u/a -3
MR u/a -1
SO u/a -1
To measure on next visit.

14.  Impression:


Bilateral astigmatism. Vision improves with spectacles
Rx.
Intermittent small XT on LE. Patient able to control.
 Management


and plan:
Prescribe glasses.
TCA 3/12 to monitor binocular assessment on next visit
(NPC, CT, stereopsis, monitor squint) and to review on
general clinic.

15. SECOND FOLLOW-UP VISIT
Date: 9th July 2012 (OPTOM clinic)
CHIEF COMPLAINT
-Come today for RA and BV assessment.
OCULAR HISTORY
- No active complaint with current glasses.
- Patient comply with glasses.

16. RE
LE
6/9-2
6/9+3
Current glasses
+0.25/-1.75x11
Pl/-1.25x1
Refraction
+0.25/-1.75x15
Pl/-1.25x175
VA (aided)
NPC

Reduced compared to patient’s age
Impression:
New spectacles Rx almost the same with current glasses.
 BE mod astigmatism.
 LE Intermittent small XT with convergence insufficiency. (NPC reduced
compare to pt’s age).


Management and plan:
Continue wearing glasses constantly.
 To start pen-to-nose visual therapy ( 5session, 5x per day).
 TCA 3/12 to monitor binocular assessment on next visit (NPC, CT,
stereopsis, monitor squint) and to review on general clinic.


17. THIRD FOLLOW-UP VISIT
Date: 25th October 2012 (OPTOM clinic)
CHIEF COMPLAINT
-Come today for RA and BV assessment.
OCULAR HISTORY
- No active complaint with current glasses.

18. RE
LE
6/7.5+3
6/7.5-1
Current glasses
+0.25/-1.75x10
Pl/-1.25x175
Refraction
+0.25/-1.75x15
Pl/-1.25x170
VA (aided)
AHP
Hirschberg
Cover test
Absent
Symmetry
(33cm) : Orthophoria
(6m) : Small exophoria with fast recovery
OMT
RMR o/a.
NPC
Reduced compared to patient’s age

19.  Impression:





No significant changes in Rx.
BE mod astigmatism.
LE Intermittent small XT with convergence insufficiency.
(NPC reduced compare to pt’s age).
RE medial rectus overaction.
No squint noted at primary gaze.
 Management



and plan:
Continue wearing glasses constantly.
To start pen-to-nose visual therapy ( 5session, 5x per day)
to improve NPC.
TCA 3/12 to monitor binocular assessment on next visit
(To review on VA, RA and squint assessment).

20. FOLLOW UP VISIT
IMPRESSION
MANAAGEMENT & PLAN
4TH FOLLOW UP VISIT
(22/4/2013)
 BE astigmatism
LE slightly exotropia with
BV good with Rx
• Continue wearing current
glasses
• Continue pen-to-nose
exercise (5session/5x/day)
•KIV dot card exercise @
Brock’s string if convergence
still reduced.
• TCA 3/12 to review RA and
NPC.
5TH FOLLOW UP VISIT
(24/7/2013)
 RE hyperope and
astigmatism. Flat cornea from
k-reading?
Poor convergence, poor
prognosis as suppress RE
during previous therapy.
•Continue wearing current
glasses
• Continue pen-to-nose
exercise coupled with
physiological diplopia
awareness.
• TCA 3/12 to review RA and
NPC.
6TH FOLLOW UP VISIT
(3/11/2013)
 BE low refractive error.
NPC improves slightly.
•Continue wearing current
glasses
• Continue pen-to-nose
exercise coupled with
physiological diplopia
awareness.
• TCA 4/12 to review RA and
NPC.
V
I
S
I
T
S
U
M
M
A
R
Y

21.  Clinical
1)
2)
3)
4)
5)
6)
investigations:-
Age of presentation
Visual acuity
Abnormal head posture (AHP)
Cover test
Ocular motility
Hess chart

22.  History



taking
During history taking, we need to ask about the age of
presentation.
Because this syndrome may be occurs during first year
of life, but occasionally later.
Treatment need to give as early as possible for better
prognosis of vision and patient’s condition.

23. Visual acuity
Visits
RE
 Visual


2/2/2012
acuity
Visual acutiy is good
if BSV maintained and
no anisometropia and
amblyopia occur.
Important to detect
and treat the
underlying causes
within plastic ages to
avoid development of
amblyopic eyes.
LE
6/24
6/12
23/4/2012
6/12
6/7.5
9/7/2013
6/9-3
6/9+3
25/10/2013
6/7.5+3
6/7.5-1
22/4/2013
6/6-2
6/6-1
24/7/2013
6/6-1
6/6-1
3/11/2013
6/6-1
6/6-1
* This patient good vision prognosis because he not
developed amblyopic eyes.

24.  Abnormal
Head
Posture (AHP)


Face turn to affected
side in types A and B.
Face turn to unaffected
side in type C.
For this patient, the
examiner detected
patient had face turn to
the left (affected side).
Visits
AHP
23/4/2012
Sometimes chin down
3/11/2013
Face turn to the left
and chin up in primary
position slightly.

25.  Cover




test
BSV often maintained with AHP.
Without AHP, types A and B – esotropia. Type C –
exotropia.
In this patient all clinical findings are showed patient
had slightly exo deviation.
Probably had type 3? Confused and indistinct
classification.

26.  Ocular







motility
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
Possible defect of convergence
Strabismus
 May have updrift or downdrift on adduction with A or V
pattern
Amblyopia if not adopted AHP

27. 
Hess screen test



Additional test- Hess chart: to investigate incomitant strabismus
in order to asses paretic element .
May show a large restriction for a small deviation in primary
position.
Alternative may use binocular Bjerrum visual field.
Examples of Hess screen chart result among Duane’s Syndrome Type 1.

28. Type A
Abduction and
adduction are
both defective
but abduction
is more
defective than
adduction.
Optometric impression Type B
follows Lyle’s
classification.
 Patient had LE
Duane’s retraction
Type C
syndrome Type A.
Defective
abduction only,
adduction is
normal.
 Optometric
impression

Abduction and
adduction are
both defective,
but adduction is
more defective
than abduction.

29. 1. Correction of
refractive error
 Optometric
management and plan

Management in this
case: Correction of
refractive error 
observation  BV
therapy.
2. Occlusion
therapy if
amblyopia present
3. Observation
4. Surgery indicated
if:
• Marked AHP
• Decompensating
• Cosmetically poor
deviation
• Diplopia occuring
more frequent

30.  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.

31.  Kim
JH, Hwang JM. Presence of the abducens
nerve according to the type of Duane's
retraction syndrome. Ophthalmology. 2005
Jan;112(1):109-13. PubMed PMID: 15629829.
 National Human Genome Research Institute
(http://www.genome.gov/11508984)
 Dr. Norliza Lecture Notes on Nonstrabismic
anomaly.
 Lecture notes Bengkel penglihatan Binokular dan
Terapi Visual Tahap 1/2013.
 E-His systems, Hospital Sultanah Bahiyah, Alor
Setar.

32.  Special
thanks to all clinician optometrist in
Sultanah Bahiyah Hospital.
 Special thanks to Ms Nur Shafiah ( Supervisor’s
optometrist) for this case.