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Duane retraction syndrome
1. Dr. K. Vasantha M.S.,F.R.C.S., Edin
Director, RIO, Chennai. (Rtd)
2. Duane retraction syndrome is one of the congenital
cranial dysinnervation disorders.
It is an ocular motility disorder.
There is defective horizontal movement of the eye along
with narrowing of the palpebral fissure on adduction and
widening on abduction
Usually unilateral with left eye more commonly affected
Slightly more common in females
3. Usually sporadic
Familial occurrence noticed
In cases of autosomal dominant inheritance with poor
penetrance, CHN1 is found as the gene responsible
Problems in this gene affect the axons of the Abducent
nerve and Oculomotor nerve
Rarely recessive also
Deletions are seen in chromosome 1,4,5 and 8.
4. Type 1: Most common type. Abduction is limited more
than adduction with little innervation from VI nerve.
Lateral rectus is paradoxically innervated by III nerve.
Type 2: Limitation of adduction with exotropia in the
primary position. Here also retraction and narrowing of
palpebral fissure occurs on adduction.
Type 3: Both adduction and abduction are affected.
Globe retraction and fissure narrowing occur on
adduction as usual. There is simultaneous innervation of
medial and lateral recti.
5. Type 4: There is abduction on attempted adduction as
the third nerve supplies the lateral rectus and not the
medial. Here there will be gross exotropia
One must always note whether there is orthophoria,
exotropia or esotropia as surgical corrections will be
based on the type of tropias.
6. V pattern strabismus is more common than A
Narrowing of the fissure is due to co contraction of
medial and lateral recti
Though the sixth nerve nucleus is affected the
interneurons to the medial rectus is not affected
7. Ortho, eso or exophoria
Anisocoria
Heterochromia
Ptosis
Congenital cataract
Face turn
8. In Duane radial ray syndrome radial dysplasia causing a
range of limb anomalies like thumb hypoplasia to
absence of limbs can occur – Okihiro syndrome
With autosomal recessive type deafness, facial
weakness, vascular malformations and mental
retardation has been noticed.
Goldenhar syndrome
9. Holt- Oram syndrome in which Duane is seen along with
Horner’s syndrome, keratoconus, morning glory disc and
cardiac abnormalities
Wildervanck syndrome – nystagmus, coloboma and limb
abnormalities. Cervico- oculo-acoustic syndrome.
Hearing loss due to inner ear abnormality, fusion of
cervical vertebrae causing a short, web neck (Klippel-
Feil anomaly) and Duane. Dermoid, subluxation of lens
and pseudopapilledema also may be seen
10. Klippel Fiel syndrome with micro cornea, Marcus Gunn
jaw winking and optic nerve hypoplasia
Oculo cutaneous albinism
Fetal alcohol syndrome
11. Seen in cases of trauma
Orbital tumors
Dipolpia will be present in these cases which helps us to
differentiate it from true Duane
Sixth nerve palsy is a differential diagnosis
12. Depends on
Any deviation in the primary position
If esotropia is present accommodation excess must be
ruled out and glasses must be prescribed
Change in head posture
Severe globe retraction and/or upshoot
Severity of restricted movements
Amblyopia
13. Esotropia with Duane - FDT is done to see if the medial
rectus is tight. Then up to 5 mm medial rectus recession
has to be done to correct 20 prism diopters of tropia.
Eso more than 20 PD - contra lateral medial rectus has to
be resected.
Exotropic Duane can be managed with lateral rectus
recession. For large tropias fixation of lateral rectus to
the periosteum and vertical muscle transposition can be
done
14. When dealing with more than two muscles anterior
segment ischemia must be kept in mind
Resection must be avoided as for as possible as it may
further restrict the movement
15. For globe retraction both co-contracting muscles i.e.
medial and lateral recti must be recessed
For over shoots – Y splitting of the lateral rectus along
with recession is done. One arm of the muscle is sutured
slightly up and the other slightly down so that counter
action during elevation and depression will prevent over
shoots.