Duane

syndro… EN
Introduction

Duane syndrome
Presentation
Causes
Genetics Connected to: Strabismus Ophthalmology Human eye

Diagnosis
Classi cation From Wikipedia, the free encyclopedia
Di erential diagnosis
Duane syndrome is a congenital rare type of strabismus most commonly Duane's syndrome
Treatment
characterized by the inability of the eye to move outwards. The syndrome Other names DRS[1]
Epidemiology was rst described by ophthalmologists Jakob Stilling (1887) and
See also Siegmund Türk (1896), and subsequently named after Alexander Duane,
who discussed the disorder in more detail in 1905.[2]
References
Further reading Other names for this condition include: Duane's retraction syndrome, eye
retraction syndrome, retraction syndrome, congenital retraction
External links
syndrome and Stilling-Türk-Duane syndrome.[3]

Presentation
The characteristic features of the syndrome are:

Limitation of abduction (outward movement) of the a ected eye.

Less marked limitation of adduction (inward movement) of the same
eye.
Retraction of the eyeball into the socket on adduction, with associated Duane Syndrome type I in left eye. 10-year-old
narrowing of the palpebral ssure (eye closing). girl.
Widening of the palpebral ssure on attempted abduction. (N. B. Mein Specialty Ophthalmology 
and Trimble[4] point out that this is "probably of no signi cance" as the
phenomenon also occurs in other conditions in which abduction is
limited.)
Poor convergence.
A head turn to the side of the a ected eye to compensate for the
movement limitations of the eye(s) and to maintain binocular vision.

While usually isolated to the eye abnormalities, Duane syndrome can be Patient with Duane syndrome attempting to
associated with other problems including cervical spine abnormalities look far right. Notice the a icted left eye faces
Klippel-Feil syndrome, Goldenhar syndrome, heterochromia, and congenital straight and up, rather than following the right
eye to the right.
deafness.[5]

Causes
Duane syndrome is most probably a miswiring of the eye muscles, causing some eye muscles to contract when they
shouldn't and other eye muscles not to contract when they should.[3] Alexandrakis and Saunders found that in most
cases the abducens nucleus and nerve are absent or hypoplastic, and the lateral rectus muscle is innervated by a branch
of the oculomotor nerve.[6] This view is supported by the earlier work of Hotchkiss et al. who reported on the autopsy
ndings of two patients with Duane's syndrome. In both cases the sixth cranial nerve nucleus and nerve was absent, and
the lateral rectus muscle was innervated by the inferior division of the third or oculomotor nerve. This misdirection of
nerve bres results in opposing muscles being innervated by the same nerve. Thus, on attempted abduction,
stimulation of the lateral rectus via the oculomotor nerve will be accompanied by stimulation of the opposing medial
rectus via the same nerve; a muscle which works to adduct the eye. Thus, co-contraction of the muscles takes place,
limiting the amount of movement achievable and also resulting in retraction of the eye into the socket. They also noticed
2
mechanical factors and considered them secondary to loss of innervation: During corrective surgery brous
attachments have been found connecting the horizontal recti and the orbital walls and brosis of the lateral rectus has
been con rmed by biopsy. This brosis can result in the lateral rectus being 'tight' and acting as a tether or leash. Co-
contraction of the medial and lateral recti allows the globe to slip up or down under the tight lateral rectus producing
the up and down shoots characteristic of the condition.[7]

Genetics
There are two known genetic associations with Duane Syndrome In some families the condition is associated with
variants in the CHN1 gene[8] and in others it is associated with variants in the MAFB gene.[9]

Diagnosis
Classi cation
Duane's syndrome has three variants:

Type I: Limited abduction with or without esotropia

Type II: Limited adduction with or without exotropia
Type III: Limitation of both abduction and adduction and any form of horizontal strabismus

The rst type is more common and accounts for 85% of the cases.[5][10]

Di erential diagnosis
In the clinical setting, the principal di culties in di erential diagnosis arise as a consequence of the very early age at
which patients with this condition rst present. The clinician must be persistent in examining abduction and adduction,
and in looking for any associated palpebral ssure changes or head postures, when attempting to determine whether
what often presents as a common childhood squint (note-"squint" is a British term for two eyes not looking in the same
direction[11]) is in fact Duane syndrome. Fissure changes, and the other associated characteristics of Duane's such as up
or down shoots and globe retraction, are also vital when deciding whether any abduction limitation is the result of
Duane's and not a consequence of VI or abducens cranial nerve palsy.

Acquired Duane's syndrome is a rare event occurring after peripheral nerve palsy.[12]

Treatment
The majority of patients remain symptom free and able to maintain binocularity with only a slight face turn. Amblyopia
is uncommon and, where present, rarely dense. This can be treated with occlusion, and any refractive error can also be
corrected.

Duane syndrome cannot be cured, as the "missing" cranial nerve cannot be replaced, and traditionally there has been
no expectation that surgery will result in any increase in the range of eye movement. Surgical intervention, therefore,
has only been recommended where the patient is unable to maintain binocularity, where they are experiencing
symptoms, or where they are forced to adopt a cosmetically unsightly or uncomfortable head posture in order to
maintain binocularity. The aims of surgery are to place the eye in a more central position and, thus, place the eld of
binocularity more centrally also, and to overcome or reduce the need for the adoption of an abnormal head posture.
Occasionally, surgery is not needed during childhood, but becomes appropriate later in life, as head position changes
(presumably due to progressive muscle contracture).[citation needed]

Surgical approaches include:

Medial rectus recession in the involved eye or both eyes. By weakening the medial rectus muscles this procedure
improves the crossed-eye appearance but does not improve outward eye movements (abductions).[citation needed]
Morad et al. showed improved abduction after modest unilateral medial rectus recession and lateral rectus resection
in a subgroup of patients with mild eye retraction and good adduction before surgery.[13]
Lateral transposition of the vertical muscles described by Rosenbaum has been shown to improve range of
movement of the eye. The surgical procedure produces 40-65 degrees of binocular eld. Orbital wall xation of the
lateral rectus muscle (muscle is disinserted and reattached to lateral orbital wall) is recommended an e ective
method to inactivate a lateral rectus muscle in cases of marked anomalous innervation and severe cocontraction.[14]

Epidemiology
Most patients are diagnosed by the age of 10 years and Duane's is more common in girls (60 percent of the cases) than
boys (40 percent of the cases). A French study reports that this syndrome accounts for 1.9% of the population of
strabismic patients, 53.5% of patients are female, is unilateral in 78% of cases, and the left eye (71.9%) is a ected more
frequently than the right.[15] Around 10–20% of cases are familial; these are more likely to be bilateral than non-familial
Duane syndrome. Duane syndrome has no particular race predilection.[citation needed]

See also

References

Further reading

External links

Diseases of the human eye (H00–H59360–379)

Categories

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Cover photo is available under CC BY-SA 3.0 license. Credit: Jmarchn (see original le).

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