Duane’s Syndrome
Violent Violation of
Sherrington’s Law
Definition
Disturbance of ocular movement
characterized by simultaneous
contraction of the medial and lateral
rectus muscles in adduction
History
1879
1887
1895
1896
1899
1900
1905
- Heuck describes a case of retraction in adduction
- Stilling
- Sinclair
- Bahr
- Turk
– Wolff
- Duane presents 54 collected cases
Duane's known as Stilling-Turk-Duane Syndrome in Europe
Prevalence
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Types I, II, and III
Incidence 1-4 percent of all strabismus
Female 54-62%
Left eye 60-75% where unilateral
Bilateral 18-22%
Many associated congenital anomalies
Occasionally familial
Diagnostic Features
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Reduced abduction
Retraction of the globe on adduction
Co-contraction of the lateral and medial
recti on adduction
Associated Features
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Upshoot or downshoot in adduction
Narrowing of palpebral fissure - minimal in
some cases
Low angle esotropia or exotropia
Head turn for fusion
"Y" or "V" pattern
Synergistic divergence
Differential Diagnosis
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Abducens palsy - usually larger angle esotropia
in primary gaze
Ocular myasthenia
Spasm of the near reflex
Medial rectus entrapment with medial orbit wall
fracture
Strabismus fixus
Ocular neuromyotonia
Graves ophthalmopathy
Duane’s-Associated Syndromes
33% of All Duane’s
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Klippel-Feil Anomaly 3-4%
Labyrinthine deafness 8-16 %
Wildervanck Syndrome both of above
Goldenhar Syndrome
Crocodile tears
Arthrogryposis multiplex congenita
Marcus-Gunn Jaw Winking Syndrome
Many others
Etiology
Neuroanatomy
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1. Deficient innervation of lateral rectus
2. Innervation of lateral rectus by anomalous
branch of 3rd nerve
3. Brainstem origin
Embryology
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1. Teratogenesis at 8 weeks gestation
2. Absence of abducens motor neurons
Type I Duane’s
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Most Common – 78%
Very reduced abduction
Globe retraction with attempted adduction
Narrowing of palpebral fissure with
adduction
Typically esotropic
Absent sixth nerve nucleus
Duane’s Retraction Syndrome
Type I Duane’s EMG
MR – Adduction
+
LR – Adduction
+
MR- Abduction
LR – Abduction
-
Type II Duane’s
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Least common -7%
Fair abduction
Reduced adduction
Globe retraction and narrowing of
palpebral fissure with adduction
Often Exotropic
Type II Duane’s EMG
MR – Adduction
+
LR – Adduction
+
MR- Abduction
LR – Abduction
+
Type III Duane’s Syndrome
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Incidence about 15%
Poor abduction and adduction
Globe retraction and narrowing fissure in
adduction
Minimal deviation in primary gaze
Tonic firing of horizontal rectus muscles
Type III Duane’s EMG
MR – Adduction
+
LR – Adduction
+
MR- Abduction
+
LR – Abduction
+
Secondary Effects of Duane’s
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Pseudo-overaction of inferior oblique
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Due to leash effect of contracting LR
V, Y and X patterns
Face turn
Treatment of Duane’s
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Rationale for treatment
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Disruptive head turn
Diplopia (rare)
Suppression and amblyopia (uncommon)
Large angle deviation in primary gaze
Deviation in up or downgaze
Treatment modalities
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Many cases require no intervention
Prism in spectacles
Surgery
Surgery-Type I
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For minimal co-contraction do large
ipsilateral MR recession
For severe co-contraction-small ipsilateral
MR recession and large contralateral MR
recession
Avoid lateral rectus resection
Approach transposition with caution
because of vertical deviations
Recession
MEDIAL RECTUS
Transposition
Surgery for Type II
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Ipsilateral lateral rectus recession
Contralateral medial rectus resection
Surgery for Type III
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Fadenoperation on Contralateral medial
rectus and lateral rectus
Surgery for Upshoot or Y-pattern
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Y-splitting of lateral rectus
Fadenoperation of lateral rectus
Bilateral Duane’s
Danger of consecutive XT
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Simultaneous recession of medial and
lateral rectus
M.R
FIN