Duane Syndrome
Description
- 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
Type | Abduction | Adduction | Deviation | Frequency |
---|---|---|---|---|
I | Poor | Normal | Ortho- or Esotropia | 70% |
II | Normal | Poor | Ortho- or Exotropia | 15% |
III | Poor | Poor | Ortho-, Eso- or Exotropia | 15% |
Clinical Characteristics
- 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:
Female | Male | OS | OD | OU |
---|---|---|---|---|
58% | 42% | 59% | 23% | 18% |
- Gender and Laterality in 835 cases of Duane Syndrome. DeRespinis PA, Wagner RS, Guo S; Duaneās Retraction Syndrome. Surv Ophthalmol 38:257, 1993
Exam Findings
- 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%
Selected Associated Syndromes
- 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)
Treatment
- 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