Duane syndrome, also called Duane retraction syndrome (DRS), is a congenital and non-progressive type of strabismus due to abnormal development of the 6th cranial nerve. It is characterized by difficulty rotating one or both eyes outward (abduction) or inward (adduction). On the other hands Duane Retraction Syndrome is a congenital strabismus syndrome occurring in isolated or syndromic forms. It presents with a variety of clinical features including diplopia, anisometropia, and amblyopia.

1. Duane's Retraction Syndrome (DRS)
MD.AZIZUL ISLAM
Associate Optometrist (IIEI&H)

2.  Duane syndrome, also called Duane retraction syndrome (DRS), is a
congenital and non-progressive type of strabismus due to abnormal
development of the 6th cranial nerve.
 It is characterized by difficulty rotating one or both eyes outward
(abduction) or inward (adduction).
 On the other hands Duane Retraction Syndrome is a congenital
strabismus syndrome occurring in isolated or syndromic forms. It
presents with a variety of clinical features including diplopia,
anisometropia, and amblyopia.
Duane's Syndrome/DRS

3.  This syndrome was first described by Stilling (1887) and Turk (1896)
and is also known as the Stilling-Turk- Duane’s syndrome.
 Duane (1905) discussed the disorder in more detail and it became
generally known as Duane’s retraction syndrome.
 The retraction syndrome is a congenital disorder and has been
reported in a neonate aged just 1 day (Archer et al. 1989)
History of Duane's Syndrome

4.  Prevalence of about 1/1000 in general population
 Females (60%)
 Males (40%)
 Accounts for up to 4% of all strabismus cases
 Most common type of congenital aberrant ocular innervation
 ~70% of cases are isolated
 ~30% of cases are associated with other congenital anomalies
Epidemiology of DRS

5.  Duane syndrome is due to miswiring of nerves to the eye muscles.
 In Duane syndrome, the 6th cranial nerve that controls the lateral rectus
muscle (the muscle that rotates the eye out towards the ear) does not develop
properly. Why the nerve does not develop is not fully understood. Thus, the
problem is not primarily with the eye muscle itself, but with the nerve that
controls to the muscle.
 There can also be associated with miswiring of the 3rd cranial nerve, which
normally controls the medial rectus muscle (the muscle that rotates the eye
toward the nose).
The Cause Of DRS

6.  Amblyopia (reduced vision in the affected eye): Occurs in 10% of
patients.
 Strabismus: The eyes may be misaligned and point in different
directions some or all of the time.
 Complete or less often partial absence of abduction
 Retraction of globe on adduction
 Narrowing of palpebral fissure during adduction (induced ptosis)
 Partial deficiency of adduction
 Oblique movement with attempts at adduction
 Upshoot or downshoot of globe with adduction (Leash Phenomenon)
 Widening of palpebral aperture with abduction
 Deficiency of convergence
Clinical Features of DRS

7.  Complete, or less often partial, absence
of outward movement (abduction) of
the affected eye
 Partial, or rarely complete, deficiency
of inward movement (adduction) of
the affected eye
 Retraction of the affected eye into the
orbit when it is adducted
Clinical Features of DRS CNTD..

8.  Partial closure of the eyelids
(pseudoptosis) of the affected eye
when it is adducted
 A sharply oblique movement of the
affected eye, either down and in
(downshoot) or up and in (upshoot
), when it is adducted
 Deficiency of convergence, with the
affected eye remaining fixed in the
primary position while the other eye
is converging
Clinical Features of DRS CNTD..

9. Syndromes Associated With
Congenital Anomalies of DRS
Okihiro's syndrome: Duane syndrome and radial ray defects
Wildervanck syndrome: Duane syndrome, Klippel-Feil anomaly, and
deafness.
Moebius: Congenital paresis of facial and abducens cranial nerves.
Holt-Oram syndrome: Abnormalities of the upper limbs and heart.
Morning Glory syndrome: Abnormalities of the optic disc
Goldenhar syndrome: Malformation of the jaw, cheek and ear,
usually on one side of the face.

10. Types Of DRS

11. Papst Types Of DRS

12. Clinical View of Papst Types DRS

13.  Type I: Limited abduction with or without esotropia.
 Type II: Limited adduction with or without exotropia.
 Type III: Limitation of both abduction and adduction and any form of
horizontal strabismus.
 Brown(1950) has classified Duane's syndrome according to the
characteristics of the limitation of movement-
 Type A: With limited abduction and less-marked limitation of adduction
 Type B: Showing limited abduction but normal adduction.
 Type C: The limitation of adduction exceeds the limitation of abduction.
There is an exotropic deviation and a head turn to compensate the loss
of adduction.
Malbran & Brown Types Of DRS

14.  Image Showing Bilateral Duane
Retraction Syndrome
Left Panel: limited abduction of right
eye with induced ptosis on
adduction of left eye.
Right Panel: limited abduction of left
eye with induced ptosis on
adduction of right eye
Clinical View of Malbran Brown Types
DRS

15. There are 3 types of Duane Retraction According to Huber :
 Type 1 comprises 75-80% of patients and presents with an esotropia in
primary gaze with a compensatory head turn to the involved side.
 Type 2 comprises 5-10% of patients and presents with an exotropia in
primary gaze with a compensatory head turn to the uninvolved side.
 Type 3 comprises 10-20% of patients, can present with either an esotropia
or exotropia in primary gaze, and will have a compensatory head turn to the
involved side. The ability to adduct in this type is absent to restricted as
compared to normal to mildly restricted in types 1 and 2.
Huber Types Of DRS

16. Clinical View of Huber Types DRS

17.  Proper Orthoptic evaluation
 The cover test
 Ocular movements should be tested
 Changes in lid and globe position on
horizontal gaze should be carefully
assessed
 The deviation should be measured fixing
with each eye
 Confirmation of binocular single vision
 Hess chart
 The presence or absence of diplopia
Diagnosis Protocol of DRS

18. Documentation of DRS

19. Documentation of DRS

20. Documentation of DRS

21. Management of DRS
Spectacles or contact lenses for refractive error.
Prism glasses to improve the compensatory head position.
Treat amblyopia with standard therapy. In this case, no patching
treatment is indicated yet as vision BE is almost similar
Meridional amblyopia usually has good prognosis with spectacles alone
Botulinum Toxin: A study published in the Iranian Journal of
Ophthalmology in 2008 investigated the efficacy of injecting botulinum
toxin A as a treatment in 4 patients. The results concluded that botulinum
toxin decreases the amount of deviation and leash phenomenon
(upshoot or downshoot of globe with adduction).
Non-Surgical Management

22. Surgery cannot cure Duane Retraction
Syndrome, but it can correct for the deviation in
the primary position, thereby improving a
compensatory head position that can occur in
some individuals. It can also improve the leash
phenomenon.
Management of DRS
Surgical Management

23. References
 Clinical Ophthalmology: A Systematic Approach
by Jack J. Kanski.
 https://eyewiki.org/Duane_Retraction_Syndrome
 Picture: Books ,Google.

24. THANKS-ALL