A snapshot of Duane retraction syndrome

1. DUANE RETRACTION SYNDROME (DRS)
DR HAITHAM AL MAHROUQI
OPHTHALMOLOGY RESIDENT, OMSB (2018)

2. DRS
 A type of congenital cranial dysinnervation syndroms: Results from agenesis of a cranial nerve nucleus.
Others include congenital fibrosis of the extraocular muscles (CFEOM) and Mobius syndrome.
 Special form of strabismus where there is congenital agenesis of the sixth nerve nucleus and
innervational misdirection of part of the medial rectus nerve to the lateral rectus muscle.
 This results in limited abduction and co-contraction of the medial and lateral recti on adduction.
 The co-contraction causes the globe to retract causing lid fissure narrowing on adduction.
 Duane’s syndrome is usually sporadic without systemic manifestations, however, it can be familial or
associated with systemic disease (Goldenhar syndrome, Klippel-Feil syndrome, and intrauterine
thalidomide exposure).
 Mostly sporadic. 5-10% autosomal dominant.

3. TYPES OF DRS
 Type 1: Poor abduction (most common, accounting for 50-80% of cases)
 Type II: Poor adduction
 Type III: Poor adduction and abduction
 COUNT THE Ds!

4. TYPE 1
How to differentiate it from 6th nerve palsy?
• Profound abduction deficit and only modest esotropia.
• Globe retraction.

5. TYPE II

6. TYPE III

7. TYPES

8. TREATMENT
 Address face turn?
 Due to incommitent strabismus
 Address retraction of the globe
 Address the upshoot or downshoot: vertical slippage of a tight lateral rectus muscle by 1-2 mm

9. FACE TURN DUE TO ESOTROPIA IN DRS 1
 Type 1 esotropia: Medial rectus
recession only. Lateral rectus is fibrotic.
If angle of deviation is > 20PD, recess
the other eye medial rectus.
 Type II exotropia: Lateral muscle
recession
 Type III normally have straight eyes

10. GLOBE RETRACTION
 Managed with recession of both medial and lateral recti muscle.

11. UPSHOOT OR DOWNSHOOTS
 Y split of the lateral rectus. OR
 Retroequatorial fixation of the muscle.