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Case of the week 150  ( December 2013 )
Bilateral Duane  syndrome II to III


This 3 years old girl was noted by her parents to have out deviation of the right eye from shortly after birth. As she began sitting up and walking they noted that she turns her head to the left and assumes right gaze. The child is healthy and there is no family history of strabismus.

Refraction: OD +1.50          OS +1.00


 


 See video of this patient
 


Duane syndrome

Duane syndrome is a congenital type of strabismus characterized by Limitation of abduction ( Duane type I ), limitation of adduction ( Duane type II ) or limitation of both abduction and adduction ( Duane type III ). Duane patient may be orthotropic, esotropic in type I, exotropic in type II, or any other form in type III.The other characteristic feature of Duane syndrome is retraction of the globe on attempt adduction with consequent narrowing of the palpebral fissure, the name (Duane Retraction Syndrome ) is also used in the literature. Face turn is a common finding in Duane patients, the face turns towards the direction where the eye can not go. Another feature of Duane Syndrome is the up shot or down shot of the affected eye on adduction. The syndrome was named after Alexander Duane who discussed the disorder in 1905.

Duane syndrome can be associated with other congenital anomalies including cervical spine abnormalities, Klippel-Feil syndrome, Goldenhar syndrome, heterochromia, and congenital deafness.

Duane syndrome is caused by miswiring of the eye muscles where the opposing muscles ( the medial rectus and the lateral rectus ) become innervated by the same nerve ( 3rd cranial nerve ). Thus, on attempted abduction, stimulation of the lateral rectus via the 3rd nerve will be accompanied by stimulation of the opposing medial rectus via the same nerve. Thus, co-contraction of the muscles takes place, limiting the amount of movement achievable and also resulting in retraction of the globe. Co-contraction of the medial and lateral recti during adduction may make the globe to slip up or down under the tight lateral rectus producing the up and down shoots.

   Many patients with Duane Syndrome remain symptom free and able to maintain binocularity with only a slight face turn.

     Duane syndrome cannot be cured, as the miswiring cannot be corrected, no expectation that surgery will result in any increase in the range of the limited eye movement. Surgical intervention has only been indicated when there is :

  1. ·       Uncomfortable head posture

  2. ·       Esotrpia or exotropia in the primary position

  3. ·       Up or down shoots on adduction

  4. ·       Disfiguring narrowing during adduction

  Pre op post op
Primary position
XT
looking left
Rt eye Downshoot
looking right
Lt eye narrowing

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