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Duane’s Syndrome

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

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Duane’s Syndrome

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  1. Duane’s Syndrome Violent Violation of Sherrington’s Law

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

  3. 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

  4. 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

  5. Diagnostic Features • Reduced abduction • Retraction of the globe on adduction • Co-contraction of the lateral and medial recti on adduction

  6. 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

  7. 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

  8. Duane’s-Associated Syndromes33% 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

  9. 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

  10. 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

  11. Duane’s Retraction Syndrome

  12. Type I Duane’s EMG

  13. Type II Duane’s • Least common -7% • Fair abduction • Reduced adduction • Globe retraction and narrowing of palpebral fissure with adduction • Often Exotropic

  14. Type II Duane’s EMG

  15. 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

  16. Type III Duane’s EMG

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

  18. 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

  19. 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

  20. Recession MEDIAL RECTUS

  21. Transposition

  22. Surgery for Type II • Ipsilateral lateral rectus recession • Contralateral medial rectus resection

  23. Surgery for Type III • Fadenoperation on Contralateral medial rectus and lateral rectus

  24. Surgery for Upshoot or Y-pattern • Y-splitting of lateral rectus • Fadenoperation of lateral rectus

  25. Bilateral Duane’sDanger of consecutive XT • Simultaneous recession of medial and lateral rectus M.R

  26. FIN

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