Duane syndrome

What is Duane Syndrome?

Duane syndrome, also called Duane retraction syndrome (DRS), is a congenital and non-
progressive type of strabismus due to abnormal development of the 6th cranial nerve. It is
characterized by difficulty rotating one or both eyes outward (abduction) or inward (adduction).
There may also be changes of eyelid position on attempted movement of the eyes.

Risk Factors
The only known risk factor of an isolated type of Duane Retraction Syndrome is an affected
parent, leads to a 50% chance of passing the gene onto offspring. This risk, however, is only
associated with 10% of isolated cases, as 90% of these cases occur sporadically. An affected
parent is also a risk factor for the syndromic forms; however, the chance of passing a gene onto
offspring varies according to the syndrome.
Physical Examination
Duane syndrome can be isolated, or it can be associated with other congenital anomalies.
Duane syndrome is an isolated finding in approximately 70% of patients, but it may be
associated with other malformations. Major anomalies associated with Duane syndrome can be
grouped into 4 categories: skeletal, auricular, ocular, or neural. Duane syndrome can also be
associated with other well-defined syndromes, including Okihiro syndrome, Wildervanck
syndrome, Holt-Oram syndrome, morning-glory syndrome, and Goldenhar syndrome.

Duane syndrome can be associated with both ocular anomalies and systemic anomalies. Ocular
anomalies commonly associated with Duane syndrome include dysplasia of the iris stroma,
pupillary anomalies, cataracts, heterochromia, Marcus Gunn jaw-winking, coloboma, crocodile
tears, and microphthalmos. Systemic anomalies include Goldenhar syndrome, Klippel-Feil
anomalad, and congenital labyrinthine deafness.

Most cases are sporadic. The congenital anomaly consists of absence of the abducens nerve
nucleus in the brainstem and simultaneous innervation of the affected lateral rectus muscle by
the inferior branch of the oculomotor nerve. The left eye is involved in 60% of cases. Duane
syndrome is often bilateral but asymmetrical.

Most patients with sensory adaptation demonstrate a peculiar sensory adaptation with excellent
binocular functions in directions of gaze where visual axes are aligned and usually suppression
without diplopia in the field of the paretic muscles.

Some suggest that the second image is ignored, rather than suppressed, in view of the
difficulties encountered in plotting the suppression scotoma.

Signs
Signs of Duane syndrome include the following:

• Head turn to maintain fusion (about 66% of cases, 76% of unilateral cases)

• Strabismus (about 77% of cases)

• Globe retraction

• Palpebral fissure narrowing

• Anisometropia and/or amblyopia (though not more prevalent than in the general
population)

• Heterochromia

• Iris dysplasia
• Ptosis

• Nystagmus

• Choroidal coloboma

• Hypoplasia of the optic nerve

Treatment and interventions

Any treatment is aimed at correcting a notable face turn or strabismus. Special seating
may be needed in school to accommodate a child's head turn. Special rear-view mirrors
help during driving.

A prism can be placed on the patient's glasses to correct for the face turn (though this is
not commonly used).

Vision therapy can be used to treat secondary convergence insufficiency.

Surgery

The success rate in eliminating an abnormal head position is 79-100%. Results are
stable for at least 8.75 years after surgery. Surgery does not improve motility. Surgery
does not improve stereo or fusion. A risk of diplopia may be present with or without
surgery.

The goal of surgery is to correct the face turn, decrease upshoots and downshoots,
eliminate globe retraction, and align the eyes in primary position.

Surgery can be done any time, but it is usually deferred until the child can walk.

Consider the patient's motor development (eg, ability to walk or catch a ball).

Types of surgery are as follows:

• Duane syndrome type 1: Recession of the medial rectus (no effect on abduction),
posterior fixation suturing on the normal medial rectus (may diminish adduction
innervation slightly to the involved eye, decreasing the esodeviation), or resection
involving the lateral rectus (but may turn type 1 into a severe type 2).

• Duane syndrome type 2: Recession of the lateral and medial rectus muscles in
the involved eye (may need to recess the contralateral lateral rectus if deviation or
retraction is severe), or medial rectus recession on the affected side. [22]
• Duane syndrome type 3: Recession of the medial and lateral rectus muscles for
retraction or an upshoot or downshoot (best for severe cases), Y split of the lateral
rectus can reduce retraction upshoot or downshoot, or posterior fixation suturing on the
lateral rectus reduces upshoot and downshoot.

• Duane syndrome type 4: Recession of both lateral rectus muscles (larger amount
on the involved side than on the uninvolved side), or surgery designed for the unique
patient with rare types.