Table of Contents
Duane Syndrome
Description
Clinical Characteristics
Exam Findings
Selected Associated Syndromes
Treatment
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
strabismus
,
syndrome