Duane’s Syndrome

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