Duane's retraction syndrome is caused by congenital miswiring of the medial and lateral rectus muscles. It is characterized by limited eye movement, specifically poor abduction and sometimes adduction, as well as retraction of the eyeball on attempted adduction. Management involves non-surgical options like glasses or botulinum toxin injections as well as surgical procedures to correct strabismus and improve head posture. Brown syndrome is another congenital motility disorder where the eye has limited elevation in adduction, thought to be caused by an inelastic or tight superior oblique tendon. It is typically managed surgically with tendon lengthening procedures.

1. DUANES RETRACTION
SYNDROME

2. INTRODUCTION
Forms a part of a group of conditions called
CONGENITAL CRANIAL DYSINNERVATION
DISORDERS (CCDD) which occur due to result of
developmental errors in the innervation of ocular and
facial muscles

3. Congenital miswiring of the medial and
lateral rectus muscles

4.  Described by Stilling in 1887 and Turk in 1896
Hence in European literature - referred to as the
Stilling-Turk-Duane syndrome after it’s early
describers.
 In 1905, Duane emphasized that the retraction of
the globe is an essential clinical feature of this
syndrome, thus the term “Duane’s Retraction
Syndrome” is deeply entrenched

5. PATHOGENESIS
 1. Myogenic Theory:
 This theory, suggested by earlier studies, indicates
there is fibrosis or inelasticity of the lateral rectus
muscles and that the medial rectus muscle inserts
abnormally far posteriorly.
Andrews, Caroline V., David G. Hunter, and Elizabeth C. Engle. Duane Syndrome. GeneReviews.
http://www.ncbi.nlm.nih.gov/books/NBK1190/. [July 14, 2012].

6.  2. Neurogenic Theory:
 A disturbance in embryologic development
between weeks 4-8 results in an absent abducens
nerve with anomalous innervations of the
lateral rectus muscle by a branch of the
oculomotor nerve.
Simultaneous activation of the medial and lateral
rectus muscles, as demonstrated by EMG studies,
may be the cause of global retraction
Hotchkiss, Mary G., Neil R. Miller, Arthur W. Clark, and William R. Green . Bilateral Duane's
Retraction Syndrome A Clinical-Pathologic Case Report. Arch Ophthalmol. 1980; 98: 870-874

7. TYPE 1
-Poor abduction,
good adduction
-Agenesis of 6th
nerve
-3rd nerve split
innervate LR, MR
-adduction intact
as Most of the
nerve goes to MR
TYPE 2
-Poor adduction,
good abduction
-6th nerve intact
-3rd nerve split
innervate LR, MR
-Poor adduction as
LR contract
against MR
TYPE 3
-Poor adduction,
poor abduction
-6th nerve agenesis
-3rd nerve split
innervate LR, MR
-The split is equal
TYPE 4
-Paradoxical
abduction on
attempt adduction
-6th nerve agenesis
-3rd nerve split
innervate LR, MR
Most of the nerve
innervates LR
-when ADD it ABD

8. CLASSIFICATION (HUBERS)
Type I the most common, is characterized by:
• Limited or absent ABDUCTION.
• Normal or mildly limited adduction.
• In the primary position, straight or slight esotropia.
Type II, the least common, is characterized by:
ADDUCTION.
• Limited
• Normal or mildly limited abduction.
• In primary position, straight or slight exotropia.
Type III is characterized by:
ADDUCTION AND ABDUCTION.
• Limited
• In the primary position, straight or slight esotropia.

9. Duane’s Syndrome Type I: OS
limited abduction,
retraction in adduction
G.Vicente

10. Duane’s Syndrome Type II: OS
limited adduction
retraction in adduction
G.Vicente

11. Duane’s Syndrome Type III: OS
limited adduction and abduction
retraction in adduction
G.Vicente

12. EPIDEMIOLOGY
 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
 80% of cases occur unilaterally, with a LE
predominance *
*Murillo-Correa, Claudia E., Veronica Kon-Jara, Elizabeth C. Engle, and Juan C. Zenteno. Clinical features
associated with an I126M α2-chimaerin mutation in a family with autosomal dominant Duane retraction
syndrome. J AAPOS. 2009; 13 (3): 245-248.
National Human Genome Research Institute. Learning About Duane Syndrome. Genome.gov.
http://www.genome.gov/11508984

13.  70% of cases are isolated
30% of cases are associated with other congenital
anomalies
 1. ISOLATED CASES
 90% occur sporadically and are commonly
unilateral
 Remaining 10% are inherited and these are
commonly bilateral


14.  2. SYNDROMIC FORMS
 Okihiro's syndrome: Duane syndrome and radial
ray defects
 Goldenhar syndrome: malformation of the jaw,
cheek and ear, usually on one side of the face
 Wildervanck syndrome: Duane syndrome,
Klippel-Feil anomaly, and deafness
 Moebius syndrome : 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

15. CLINICAL FEATURES
 Characterized by some or all of the following :
 1) Complete, or less often partial, absence of
outward movement (abduction) of the affected
eye

16. 2) Partial, or rarely complete, deficiency of
inward movement (adduction) of the affected
eye

17.  3) Retraction of the affected eye into the orbit
when it is adducted

18.  4) Partial closure of the eyelids (pseudoptosis) of
the affected eye when it is adducted

19.  5) A sharply oblique movement of the affected
eye, either down and in (downshoot) or up and in
(upshoot ), when it is adducted

20.  6) Deficiency of convergence, with the affected
eye remaining fixed in the primary position while
the other eye is converging

21.  7) Abnormal head posture is adopted to search for
the direction of gaze where there is no misalignment
of the two eyes in order to obtain binocularity.
Longstanding torticollis (since birth) leads commonly
to craniofacial asymmetry

22.  Strabismus: 76% of individuals have frank
strabismus in primary gaze
 Poor Binocular Vision
 Reviews of DRS patients have shown
hypermetropia of greater than +1.50 in 71% of the
patients
 Amblyopia occurs in about 10% of individuals
and will respond to standard therapy if detected
early
 Anisometropia

23. D/D
 Congenital sixth nerve palsy
 Infantile esotropia
 Mobius syndrome

24. MANAGEMENT
A) NON SURGICAL
 Spectacles or contact lenses for refractive error
 Prism glasses to improve the compensatory head
position
 Treat amblyopia with standard therapy
 Botulinum Toxin: botulinum toxin decreases the
amount of deviation and leash phenomenon
(upshoot or downshoot of globe with adduction).

25. B) SURGICAL
The aims of surgery are:
 To correct a manifest strabismus
 To centralize the field of binocular single vision,
 To overcome or reduce the need for a large
compensatory head posture
 AVOID LATERAL RECTUS RESECTION

26. Surgery is indicated for the following
reasons:
 Decompensation, giving rise to manifest
strabismus
 Abnormal head posture
 Severe globe retraction with or without up-
shoot and down-shoot

27.  For types 1 and 3 with head turn: recession of
medial rectus muscle or horizontal transposition of
vertical rectus muscles
 For types 1 and 3 with Leash Phenomenon
and/or severe globe retraction: recession of both
medial and lateral rectus muscles with possible Y-
splitting of the lateral rectus muscle
. Kekunnaya R, Kraft S, Rao V, Velez F, Sachdeva V, Hunter D. Surgical management of strabismus in
Duane retraction syndrome. Journal of American Association for Pediatric Ophthalmology and Strabismus.
2015;19(1):63-69.

28.  For type 2 with head turn and fixation with
uninvolved eye: recession of ipsilateral lateral
rectus muscle
 For type 2 with head turn and fixation with
involved eye: recession of contralateral lateral
rectus muscle
 For type 2 with Leash Phenomenon: recession
of lateral rectus muscle with possible Y-splitting

29. Complications of Surgery
 Undercorrection of primary position esotropia and
the compensatory head turn
 Overcorrection leading to secondary exotropia
 New vertical deviations can occur after vertical
rectus transposition procedures

30. BROWNS SYNDROME
 In 1950, Harold W. Brown first published on an unusual
motility disorder, characterized by limited ocular
elevation in adduction
 Brown attributed the limited elevation to a short or tight
anterior superior oblique tendon sheath. He termed this as
superior oblique tendon sheath syndrome
 He further hypothesized that the short tendon sheath was
due to a complete congenital paresis of the ipsilateral
inferior oblique muscle and secondary to sheath
contracture.

31.  In the mid 1970s, Parks and colleagues reported that a tight
tendon sheath was not the cause of Brown syndrome;
instead, it was a tight or short superior oblique tendon.
 Subsequent studies confirmed the cause of the syndrome to
be a tight or inelastic superior oblique tendon

32. ETIOPATHOGENESIS
 Brown syndrome can be divided into congenital and acquired.
 CONGENITAL BROWN SYNDROME
 Superior oblique Brown syndrome
 A tight or inelastic superior oblique tendon muscle complex
would restrict ocular elevation in adduction. Many theories for
the cause of the tight or inelastic tendon exist.

33.  Helveston theory of abnormal telescoping
mechanism
 Earlier , it was believed that the superior oblique tendon moves
through the trochlea much like a rope through a pulley.
 Through a detailed anatomical study, Helveston showed that the
superior oblique tendon appears to move in part by a
telescoping or slide-by fashion with only the central fibers of
the tendon making the total excursion
 Congenital Brown syndrome could be caused by a
developmental abnormality of the elastic-crossed fibers that
normally allow the telescoping movement of the central tendon
fibers.

34. Helveston EM, Merriam WW, Ellis FD, Shellhamer RH, Gosling CG. The trochlea.A study of the
anatomy and physiology. Ophthalmology. 1982 Feb. 89(2):124-33

35.  Wright hypothesis of congenital inelastic superior oblique
muscle-tendon complex
 In 1999, Wright described a computer simulation of Brown
syndrome, using two specific models, as follows:
 (1) a short superior oblique tendon, and
 (2) a stiff superior oblique tendon (stretched sensitivity).
 The computer model showed that a tight or inelastic muscle-
tendon complex was the best fit for the Brown syndrome
pattern of deviation.
Wright KW. Brown's syndrome: diagnosis and management. Trans Am Ophthalmol Soc. 1999. 97:1023-
109.

36.  Non superior oblique Brown syndrome
 Nonsuperior oblique Brown syndrome or pseudo-Brown
syndrome is a restriction of ocular elevation in adduction
caused by pathology other than an abnormality of the
superior oblique muscle or tendon.
 Congenital bands: Inferior orbital fibrous adhesions or
fibrous bands to the posterior globe are rare causes of
restriction of elevation in adduction.
 Congenital inferior location of lateral rectus muscle
pulley: Rare case of congenital Brown syndrome are
caused by congenital inferior location of the lateral rectus
muscle. In these cases, the limitation of elevation in
adduction is caused by the stiff lateral rectus muscle that is
congenitally located inferior to the normal position.

37.  ACQUIRED BROWN SYNDROME
 Acquired superior oblique Brown syndrome
 Abnormal telescoping mechanism: Due to one of the
following: vascular dilatation of the tendon sheath vessels and
local edema occurring within the confined area of the trochlea.

 Tight or inelastic superior oblique tendon A tight superior
oblique tendon can be caused by a mass that displaces the
tendon, a scleral buckling, or a superior oblique tendon tuck.
 A rare acquired fibrosis of the superior oblique muscle is
possibly associated with thyroid disease or an intramuscular
injection of local anesthetic,

38.  Stenosing tenosynovitis (trigger-thumb analogy theory)
 Chronic movement of the superior oblique tendon through
the trochlea can result in a traumatic tenosynovitis with
tendon-swelling and stenosis of the surrounding tendon
sheath. Trigger-thumb is a congenital or acquired
constriction or stenosis of the fibrous sheath, which
surrounds the tendon and causes secondary enlargement of
the tendon proximal to the constriction. The combination
of a sheath-stenosis and tendon swelling prevents
movements of the tendon.

39.  Superior oblique click syndrome :
 Inflammation produces a nodule on the superior oblique
tendon, just posterior to the trochlea. The nodule would
have difficulty entering the trochlea, thus restricting tendon
movement.
 Peritrochlear scarring
 Extensive scarring around the trochlea can result in
restriction of the tendon movement in both ways, resulting
in both a Brown syndrome and a superior oblique palsy
(canine tooth syndrome). This can be caused by trauma,
periocular surgery, and upper lid blepharoplasty with
removal of periorbital fat with cautery.

40.  Acquired nonsuperior oblique Brown syndrome
 Inferior orbital fibrous adhesions to the posterior globe are
caused by the following: orbital floor fracture and fat
adherence syndrome associated with inferior orbital
trauma.
 Superior nasal orbital mass: These patients usually
demonstrate a large vertical deviation in primary position
often associated with exotropia. Possible causes are a
glaucoma drainage implant or a neoplasm in the superior
orbital quadrant.

41. EPIDEMIOLOGY
 The incidence of this condition is 1 in 450 cases of
strabismus
 Approximately one in 20,000 live births.The actual
incidence may be higher as many cases are asymptomatic.
 There is equal predilection for both sexes in congenital
Brown syndrome.
 Wright noted that 5% cases are bilateral and idiopathic
Brown syndrome has a higher preponderance in females
(63%) and traumatic acquired Brown in males (82%).3

42. CLINICAL FEATURES
 A left Brown syndrome has the following
characteristics:
 1 MAJOR SIGNS
 • Usually straight with BSV in the primary
position
• Limited left elevation in adduction
• Limited left elevation on upgaze is
common
• Normal left elevation in abduction
• Absence of left superior oblique
overaction
• Positive forced duction test on elevating the
globe in adduction.
2 VARIABLE SIGNS
 • Down-shoot in adduction.
• Hypotropia in primary position.
• AHP with chin elevation and ipsilateral
head tilt

43.  Brown syndrome has also been classified as
 a) mild – no hypotropia in primary or adducted position
 b) moderate – hypotropia in adducted position
 c) severe – hypotropia in primary position
 A third classification was proposed by Jampolsky
 a) True Brown syndrome – no hypotropia in primary
 position or down gaze
 b) Brown syndrome plus – vertical deviation in primary
position or adduction ± head posture.

44. D/D
 1)Isolated inferior oblique palsy: This is characterized by
overaction of the superior oblique muscle and positive
Parks’ three step test. Forced duction test is free in inferior
oblique palsy.
 2. Double elevator palsy: Limitation of elevation is
present in both adduction and abduction. In addition the
patients have ptosis or pseudoptosis.
 3. Congenital fibrosis syndrome: The differences include
restricted elevation in abduction and esotropia on
attempted upgaze.

45.  4.Blow out fracture of the inferior orbital wall: The
elevation deficiency is more marked in abduction.
Imaging reveals a fracture and there may be associated
enophthalmos.
 5. Thyroid ophthalmopathy: The elevation deficiency is
worse in abduction than adduction.
 6. Adherence syndrome: During inferior oblique surgery
adhesions may form due to fat prolapse and limit elevation
in abduction.

47. MANAGEMENT
 The vision needs to be checked to rule out amblyopia.
 Abnormal head posture if present is indicative of the
presence of fusion.
 Forced duction testing needs to be performed to confirm
the diagnosis

48.  Spontaneous improvement is known to occur. Hence it
may be prudent to observe cases where there is no threat to
binocularity.
 Elevation in adduction exercises can improve the condition
in congenital cases or in cases where there is intermittent
Brown syndrome.
 Injection of corticosteroids has been reported to improve
the Brown syndrome in patients with inflammatory
disease.
 Systemic treatment of the underlying disease may improve
cases of acquired Brown syndrome

49.  SURGICAL MANAGEMENT
 Indications
 When there is a loss of binocularity, with the chance of
development of amblyopia and the child does not develop
an abnormal head posture, surgery is indicated.
 Mild Brown syndrome needs to be observed. If there is
presence of primary position hypotropia and
unacceptable downshoot on adduction, surgery can be
considered
 Acquired cases due to the presence of a scleral explant or a
glaucoma filtering valve need to be operated.

50.  TECHNIQUES
 Historical –Sheathectomy, Superior oblique and trochlear
luxation
 Others –
 Superior oblique tenectomy or tenotomy
 Z-tenotomy of the superior oblique
 Simultaneous inferior oblique recession
 Superior oblique tendon expander
 Chicken suture
 Superior oblique recession

53. THANK YOU