duane_syndrome

Duane Syndrome

  • Congenital abnormality of the 6th cranial nerve
    • 4th-8th week of gestation
    • Missing nucleus in most cases (4th week)
  • Abnormal innervation from 3rd cranial nerve
TypeAbductionAdductionDeviationFrequency
IPoorNormalOrtho- or Esotropia70%
IINormalPoorOrtho- or Exotropia15%
IIIPoorPoorOrtho-, Eso- or Exotropia15%
  • Most sporadic, 5% autosomal dominant inheritance
  • More common in females than males.
  • OS affected more often than OD.
  • 80% of cases are unilateral, when bilateral it is usually asymmetric.
  • Gender and Laterality:
FemaleMaleOSODOU
58%42%59%23%18%
  • Gender and Laterality in 835 cases of Duane Syndrome. DeRespinis PA, Wagner RS, Guo S; Duaneā€™s Retraction Syndrome. Surv Ophthalmol 38:257, 1993
  • Narrowing of the palpebral fissure and retraction of the globe of the involved eye on attempted adduction.
  • The amount of limitation depends on the amount of co-contraction of the medial rectus and lateral rectus.
  • Abnormal firing of the lateral rectus is found with EMG testing.
  • Angle of deviation almost always less than 30D , usually less than 15D .
  • Face turn toward the side of limited movement- permits fusion.
  • Anisometropic Ambylopia 10-30%
  • Ipsilateral congenital hearing loss 5%
  • Most cases are isolated
  • Goldenhar syndrome (hemifacial microsomia, ocular dermoids, ear abnormalities, preauricular skin tags, eyelid colobomas)
  • Wildervanck syndrome (sensorineural hearing loss, Klippel-Feil anomaly- shortness of neck with fused or missing vertebrae)
  • Treat Refractive Error
  • Consider treating Hyperopia > +2.50 if ET
  • Treat Amblyopia
  • Goals of Surgery:
    • Improve primary position alignment
    • Improve head turn
    • Decrease up-shoot or down-shoot
  • Surgical Treatment Possibilities
    • Eso-Duanes
      • Medial Rectus (MR) Recession
      • Bilateral Medial Rectus Recessions
      • Superior Rectus and/or Inferior Rectus transpositions
    • Exo-Duanes
      • Lateral Rectus (LR) Recession(s)
    • Up-shoots/Down-shoots
      • Recession with Y split or posterior fixation of LR
  • duane_syndrome.txt
  • Last modified: 2015/11/09 21:52
  • by 127.0.0.1