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 - Heuck describes a case of retraction in adduction 1887 - Stilling 1895 - Sinclair 1896 - Bahr 1899 - Turk 1900 – Wolff 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

Differential 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

Duane’s-Associated Syndromes 33% of All Duane’s         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 Duane’s       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 + MR- Abduction LR – Adduction + LR – Abduction -

Type II Duane’s      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 + MR- Abduction LR – Adduction + LR – Abduction +

Type III Duane’s 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 Duane’s EMG MR – Adduction + MR- Abduction + LR – Adduction + LR – Abduction +

Secondary Effects of Duane’s    Pseudo-overaction of inferior oblique  Due to leash effect of contracting LR V, Y and X patterns Face turn

Treatment of Duane’s   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 Y-pattern   Y-splitting of lateral rectus Fadenoperation of lateral rectus

Bilateral Duane’s Danger of consecutive XT  Simultaneous recession of medial and lateral rectus

M.R

FIN