Duane Retraction Syndrome (DRS) is a congenital strabismus characterized by co-contraction of lateral (LR) and medial rectus (MR) muscles due to abnormal innervation, leading to limited eye movement and globe retraction. The condition can be classified into three types based on the degree of eye movement limitation and typically presents with associated conditions like amblyopia and abnormal head posture. Management may include conservative approaches like correction of refractive error and surgical interventions to improve eye alignment and function.

1. Duane Retraction
Syndrome
Prepared by
Dr Robin Goh Chon Han
Ophthalmology Post-graduate
Student (UM), MBBS (IMU)
General Paediatric Department
Hospital Kuala Lumpur, Malaysia

2. Index
• Introduction
• Etiology
• Pathophysiology
• Classification
• Clinical Presentation
• Diagnosis
• Differential Diagnosis
• Management
• Reference

3. INTRODUCTION

4. DUANE RETRACTION SYNDROME
• a.k.a Stilling-Turk-Duane syndrome
• congenital strabismus syndrome characterized by co-contraction of LR & MR
due to anamalous innervation of LR during embryogenesis
• leads to variable horizontal duction deficits with narrowing of palpebral
fissure and globe retraction on attempted adduction

5. New concepts(1998 by Parsa et al)
• Congenital cranial dysinnervation disorder (CCDD)
=non-progressive developmental abnormalities of CN/nuclei with primary or secondary
dysinnervation
• Other related condition: Mobius syndrome, congenital ptosis, hereditary
congenital facial paresis

6. • 1/1000 prevalence (<5% or all forms of strabismus)
• Age of presentation is variable
• 80% sporadic
• commonly affecting female (60%)
• commonly unilateral
• left eye more common
TYPE I TYPE II TYPE III
AGE 13.5±13.46 23.00±15.62 21.93±13.04

7. Abduction deficit
(since birth)
LR inelasticity & tightness
develops with time
Globe retraction &
limitation more obvious

8. ETIOLOGY
Various theories

9. Duane
retraction
syndrome
Innervational
anomaly
theory
Neurological
anomaly
theory
Mechanical
anomaly
theory
Genetical
anomaly
theory

10. 1. Innervational
anomalies

11. 1. Innervational anomalies
• Possible embryonic insult @ 4-8wks
• leading to absent of CN 6 nerve innervation to
LR with anomalous innervation of LR by CN 3
(paradoxical innervation)
• In Abduction: NO LR electrical activity
• In Adduction: LR + MR action potentials
generated
• Co-contraction of LR & MR ----narrowing of palpebral fissue
• Synergistic innervated to SR or IR or both--upshoot /
downshoot
Normal

12. 2.
Neurological
anomalies

13. 2. Neurological anomalies
Central nervous system anomalies
• Absent of normal 6th CN innervation to LR
Lesion Example
Supranuclear Brainstem abnormality
Nuclear 6th CN nucleus hypoplasia / complete absent
Infranuclear Anamalous innervation by 3rd CN

14. • Absent of normal 6th CN innervation to LR
Supportive evidence (Synkinesis)
• Marcus Gunn Jaw Winking phenomenon
• Crocodile tears
Lesion Example
Supranuclear Brainstem abnormality
Nuclear 6th nucleus hypoplasia / complete absent
Infranuclear anamalous innervation by 3rd CN

17. 3.
Mechanical
anomalies

18. 3. Mechanical anomalies
• fibrotic changes in LR (tight & behave as inelastic band)
• or fibrotic changes in MR (tight & behave as inelastic band)
• anamalous insertion of LR & MR
• possible is a result of primary innervational anomaly
(nuclea aplasia or birth trauma)

19. 4.
Genetic
anomalies

20. 4. Genetics
• Sporadic mutation (>80%)
• PHOX2A, HOXA1, ROB03
(growth & maturation of brainstem motor neurons & axons)
• DURS1 (chromosone 2q31)
• SALL 4 gene
• if familial (10%)suspect:
CHN1 gene (autosomal dominant)
CHN1
ü autosomal dominant
ü bilateral DRS
ü assocaited vertical
anomalies
ü significant family history

21. PATHOPHYSIOLOGY

22. • Why is there limited horizontal gaze?

23. Factors affecting horizontal limitation
1) Absent or subnormal innervation to LR
2) Co-contraction of LR & MR
3) MR fibrosis & contracture that will not allow the
globe to abduct.
Keyword: Co-contraction of LR & MR

24. • What causes downshoot / upshoot?

25. UPSHOOT / DOWNSHOOT UPON ADDUCTION

26. Upshoots /
Downshoots
Bridle effect /
Leash effect
Anomalous
paradoxical
innervations
Mechanical
factors
Neurological
factors

27. • Co-contraction of MR & LR
• As globe adducts more &
moves above or below the
horizontal plane
• sudden slippage of the tight
LR causing upshoot /
downshoot
• “knife edge effect” happens in
severe case: downshoot/upshoot
happens with slightest adduction
Bridle effect /
Leash effect
COMMON IN TYPE II & III

28. Anomalous paradoxical
innervation
● Abnormal synergistic innervation between
MR and SR, IR, or SO/IO
● produce coexisting oblique muscle
dysfunction
● vertical deviation steadily increases as eye
moves from abduction to adduction
● vertical deviation in primary position

29. • What is the mechanism of globe retraction?

30. Globe
retraction in
attempted
adduction
Tight fibrotic
muscles
Anomalous
muscle slips or
foot plates to
sclera
Paradoxical
innervation (co-
contraction of MR
along with LR)

32. CLASSIFICATION

33. • No single classification is total satisfactory
• because of the wide spectrum of clinical findings
HUBER CLASSIFICATION REMARKS
TYPE I (70%) Abduction is more limited than adduction
TYPE II (7%) Adduction is more limited than abduction
TYPE III (15%) Both abduction & adduction are limited
Prevalence: Type I > Type III > Type II

34. HUBER
CLASSIFICATION
REMARKS
TYPE I (70%) Paradoxical innervation of LR by CN3, no innervation by CN6
Abduction: LR defective
Adduction: LR and MR co-contract
Limited abduction, Normal or minimally defective adduction
TYPE II (7%) LR subnormally innervaed by CN6,
also inappropriately innervated by CN 3.
Abduction: LR contracts
Adduction: LR & MR co-contract
Limited adduction. Normal abduction. Exotropia in primary position.
TYPE III (15%) Simultaneous innervation of LR & MR in primary and horizontal gaze.
Limited or complete absence of adduction and abduction
Limited or complete absence of adduction and abduction. +/- pattern strabismus
BASE ON EMG

35. CLINICAL
PRESENTATION

36. TYPE I
TYPE II
TYPE III
C/O : (From parents / child)
● Frank stabismus in primary gaze
Type I > Type III > Type II
Esotropia > Orthotropia > Exotropia
Upshoot or downshoot or both
+/- hypertropia or hypotropia in primary position
+/- Bilateral case (V > A > X pattern)
● Abnormal head position
Compensatory head posture
common in unilateral case to maintain BSV
head turn toward the side of affected eye in Type I
(esotropic Duane)

37. • Induced ptosis on adduction
passive movement of eyelids over retracting globe + decreased levator muscle action
on adduction
• Unilateral involvement
Bilateral less frequent
Bilateral involvement usually low visual acuity, less likely abnormal head posture
usually with associated congenital anomalies
• Refractive error
mostly hypermetropia / hypermetropic astigmatism
+/- Anisometropia
+/- Amblyopia (anisometropic/ametropic/strabismic/stimulus deprivation)

38. OCULAR & SYSTEMIC ASSOCIATION

39. Summary of Evaluation
• History
• Family history
• Examination include VA, head position, EOM motility, aberrant
movement
• General physical examination (TRO associated syndrome)
• Further assessment include orthoptic assessment (Refraction, EOM
motility, BSV, Accommodation)

40. GRADING OF GLOBE
RETRACTION (ADDUCTION)
COMPARED TO PALPEBRAL FISSURE HEIGHT
OF NORMAL EYE
0 no narrowing
1 <25%
2 25-50%
3 50%-75%
4 >75%

41. GRADING OF
OVERSHOOT
(ADDUCTION)
* Imaginary straight line parallel to
the intermedial canthal line is drawn
from pupillary center of fellow eye
0 Line bisects pupil of involved eye
1 Line lies between pupillary center &
pupillary margin
2 Line lies between pupillary margin &
limbus
3 Line lies at the limbus or over sclera
4 Cornea disappears below lid (pumpkin
seed sign)

42. DIAGNOSIS

43. ● Diagnosis is primarily clinical
Laboratory Test
Molecular genetic testing of CHN1 recommended only in familial case
Imaging
Not recommended for diagnostic purposes
Electromyography

44. DIFFERENTIAL
DIAGNOSIS

45. • 6th CN Palsy
• 3rd CN Palsy
• INO
• Acquired retraction syndrome (pseudoduane syndrome)
= limited abduction along with globe retraction in abduction

46. MANAGEMENT

47. • Mx depends on:
1. Primary position deviation
2. Degree of abnormal head posture
3. Severity of globe retraction and overshoots
4. Degree of limitation of ductions

48. 1. Conservative Mx
• Correct refractive error (prescribe spectacles or contact lens)
• Prism glasses to improve compensatory head position
• Treat amblyopia
2. Surgical Mx
Surgery does not eliminate fundamental innervation abnomality
but able to correct primary position deviation

49. Goals of Surgical Mx:
• Improve anomalous head posture
• Correct any angle of strabismus in primary position
• Improve globe retraction
• Expand BSV

50. General rules (AAO)
To correct ocular deviation in primary position
A. Type I = MR recession (unilateral/bilateral)
B. Type II = LR recession (unilateral/bilateral)
To correct ocular deviation & improve abduction for BSV
A. Vertical rectus transposition procedure
B. Recess-resect procedures in Duane eye
To correct globe retraction & upshoot/downshoot
A. Combined horizontal rectus recessions
B. Faden procedure on LR
C. Y splitting of LR
To correct vertical deviation
A. Vertical muscle recession

51. To correct ocular deviation in primary position
A. Type I = MR recession (unilateral/bilateral)
B. Type II = LR recession (unilateral/bilateral)

52. Patient with exotropic-Duane before and after right lateral rectus recession of 7 mm.

53. Left MR recession 6 mm done: Noted improvement of head posture and orthophoria
in primary gaze, slight improvement of abduction of the left eye

54. Right MR recession (7 mm) & left MR recession (5 mm)
for an esotropic-Duane of 25 diopters in primary gaze.
Large angle esotropia (>20PD) may need BE MR recession (contralateral eye recessed larger)

57. WHY DOING BE MR RECESSION?
Bilateral MR recession
• MR recession can improve primary position deviation
• Improve abduction of affected eye (if there was some prior lateral movement). It also
improve BSV.
• Prevent contracture of MR on affected side
Forcing Contralateral MR to work harder to maintain fixation (“fixation duress”)
MR in fixing eye receives increased innervation to maintain fixation (after op)
--> Reduced innervation to its LR (Sherrington Law)
--> Reduced innervation to MR of the affected eye (Hering Law)
--> lower risk of contracture.

58. Complications of squint surgery
• Undercorrection
• Overcorrection
• New vertical deviation
• Anterior segment ischemia
• Scleral perforation
• etc

59. Follow up for DRS
• follow up 3-6 monthly after diagnosis
(evaluate for amblyopia)
• 7-12 years old patients with good BSV can be evaluated annually or
biannually

60. Reference
• Eyewiki
• AAO
• Ramesh Kekunnya, Mithila Negalur. Duane retraction syndrome: causes,
effects and management strategies. Clinical Ophthalmology, Oct 2017.

61. Thank you
Please click the “like” button if you find this
presentation useful for you.