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Duanes Syndrome

Violent Violation of
Sherringtons Law

Definition
Disturbance of ocular movement
characterized by simultaneous
contraction of the medial and lateral
rectus muscles in adduction

History
1879 - Heuck describes a case of retraction in adduction
1887 - Stilling
1895 - Sinclair
1896 - Bahr
1899 - Turk
1900 Wolf
1905 - Duane presents 54 collected cases
Duane's known as Stilling-Turk-Duane Syndrome in
Europe

Prevalence

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

Reduced abduction
Retraction of the globe on adduction
Co-contraction of the lateral and
medial recti on adduction

Associated Features

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

Diferential Diagnosis

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

Duanes-Associated Syndromes
33% of All Duanes

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

1. Deficient innervation of lateral rectus


2. Innervation of lateral rectus by
anomalous branch of 3rd nerve
3. Brainstem origin

Embryology

1. Teratogenesis at 8 weeks gestation


2. Absence of abducens motor neurons

Type I Duanes

Most Common 78%


Very reduced abduction
Globe retraction with attempted
adduction
Narrowing of palpebral fissure with
adduction
Typically esotropic
Absent sixth nerve nucleus

Duanes Retraction
Syndrome

Type I Duanes EMG


MR Adduction

+
LR Adduction

MR- Abduction

LR Abduction

Type II Duanes

Least common -7%


Fair abduction
Reduced adduction
Globe retraction and narrowing of
palpebral fissure with adduction
Often Exotropic

Type II Duanes EMG


MR Adduction

+
LR Adduction

MR- Abduction

LR Abduction

Type III Duanes Syndrome

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 Duanes EMG


MR Adduction

+
LR Adduction

MR- Abduction

+
LR Abduction

Secondary Efects of
Duanes

Pseudo-overaction of inferior oblique

Due to leash efect of contracting LR

V, Y and X patterns
Face turn

Treatment of Duanes

Rationale for treatment

Disruptive head turn


Diplopia (rare)
Suppression and amblyopia (uncommon)
Large angle deviation in primary gaze
Deviation in up or downgaze

Treatment modalities

Many cases require no intervention


Prism in spectacles
Surgery

Surgery-Type I

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

Ipsilateral lateral rectus recession


Contralateral medial rectus resection

Surgery for Type III

Fadenoperation on Contralateral
medial rectus and lateral rectus

Surgery for Upshoot or Ypattern

Y-splitting of lateral rectus


Fadenoperation of lateral rectus

Bilateral Duanes
Danger of consecutive XT

Simultaneous recession of medial


and lateral rectus

M.R

FIN