This document discusses Duane retraction syndrome, an ocular motility disorder characterized by defective horizontal eye movement and narrowing of the eyelid when the eye adducts. It is usually unilateral and sporadic, though some familial cases are due to mutations in the CHN1 gene. There are four types described based on limitations of adduction and abduction. Duane syndrome can occur with other syndromes and limb abnormalities. Clinical features and differential diagnosis are provided. Surgical treatment depends on the type of strabismus and may include recession or resection of extraocular muscles.

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.