History

Print Version

Print Version (In Arabic)

WHAT IS DUANE SYNDROME?

Duane syndrome, also called Duane retraction syndrome (DRS), is a congenital (present since birth) and non-progressive (does not get better or worse over time) type of strabismus (eye movement problem). It is one of a group of eye diseases called the Congenital Cranial Dysinnervation Disorders (CCDDs). The eye problems in this group all have abnormal eye movement. In Duane syndrome, it can be hard to move one or both eye(s) outward toward the ear (abduction) or inward toward the nose (adduction), or both. There may also be eyelid movements when the eyes are trying to look around.

WHAT IS THE CAUSE OF DUANE SYNDROME?

Duane syndrome comes from a miswiring of nerves to the eye muscles.

In Duane syndrome, the 6th cranial nerve that controls the lateral rectus muscle (the muscle that pulls the eye out towards the ear) does not develop normally. Why the nerve does not develop well is not fully understood. Because the nerve does not develop normally, sometimes the muscle also does not develop normally, it can be weak or tight. In Duane syndrome there can also be miswiring of the 3rd cranial nerve, which normally controls the medial rectus muscle (the muscle that pulls the eye toward the nose). This is why there may be problems looking both left and right in people with Duane syndrome.

WHO GETS DUANE SYNDROME?

Most people with Duane syndrome get it spontaneously, no one else in the family has the problem. And most often only one eye is affected. Around 20% of people with Duane syndrome have problems in both eyes. For reasons unknown, the left eye is more often affected than the right eye. Duane syndrome also affects girls more often than boys. No particular race or ethnic group is more likely to get Duane syndrome. Duane syndrome is also seen with other problems that affect growth and development. 30% of people with Duane syndrome have other medical problems from birth.

WHAT is seen in people with DUANE SYNDROME?

• Strabismus: the eyes may be misaligned (point in different directions) some of the time or all of the time
• Head position: people often turn their head to one side to try to keep the eyes straight
• Amblyopia (blurry vision, weak vision, lazy eye): occurs in 10% of those with Duane syndrome
• Eyelid narrowing: the eye with Duane syndrome may look smaller than the other eye. This eyelid narrowing or squinting is easier to see when the eye moves left and right.
• Upshoot or downshoot: when looking in certain directions, the eye may sometimes roll upward or downward

IS DUANE SYNDROME CONGENITAL (PRESENT FROM BIRTH)?

YES. Duane syndrome is present from birth, even if it is not always seen during infancy. Often times, a head tilt or turn and eye movement problems can be seen in old photographs taken in early childhood for people later found to have Duane syndrome.

IS DUANE SYNDROME HEREDITARY?

Most of the time, it is not hereditary (does not run in families). In 90% of cases, there is no family history of Duane syndrome. Only 10% of people will have an affected family member and these tend to be cases where both eyes have Duane syndrome.

ARE THERE DIFFERENT TYPES OF DUANE SYNDROME?

Yes! Duane syndrome generally has three main forms. In type I Duane syndrome it is hard for the eye to move outward toward the ear. In type II Duane syndrome it is hard for the eye to move inward toward the nose. In type III Duane syndrome it is hard for the eye to move inward AND outward. Type I is the most common form of Duane syndrome. People with type I have a head turn towards the eye with problems moving and will look like they have esotropia (crossed-eye, eye turned toward nose) when trying to look straight ahead.

DO DUANE SYNDROME PATIENTS HAVE OTHER EYE PROBLEMS?

Sometimes, Duane syndrome may be seen with other eye problems including other forms of strabismus (eye movement problems), nystagmus (back-and-forth movement of the eyeball), cataract, amblyopia (blurry vision, weak vision, lazy eye), optic nerve problems, microphthalmos (abnormally small eye), and crocodile tears.

BUT, most of the time, a person with Duane syndrome is otherwise completely normal. With careful follow-up eye exams, long-term vision is usually excellent.

DO people with DUANE SYNDROME HAVE other MEDICAL PROBLEMS?

Not usually, however, some patients with Duane syndrome can have hearing problems, Goldenhar syndrome (a medical problem affecting eyes, ears, the spine and the face), and spine problems. Children with Duane syndrome in both eyes may have weakness of the muscles of the face and muscles involved in sucking  (a problem called Moebius syndrome).

WHEN IS DUANE SYNDROME TREATED with surgery?

For most patients, Duane syndrome does not require surgical treatment. Surgery for Duane syndrome may be needed for one of four reasons:

1. To make the eye turn smaller when looking straight ahead
2. To help improve a head turn
3. To help improve the eye rolling up or down when looking off to the side
4. To help improve how the eyelid looks

Surgery can also help to prevent amblyopia (weak vision or lazy eye).

HOW SUCCESSFUL IS SURGERY FOR DUANE SYNDROME?

Surgery cannot actually fix the problem of nerves that are miswired. It cannot make the nerve or muscle work “normally.” However, eye muscle surgery (strabismus surgery) can (and usually does) improve the eye movement. Some people with Duane syndrome may need multiple eye surgeries and the full effect of the surgery may take some weeks to months to show itself.

WHERE CAN I FIND MORE INFORMATION REGARDING DUANE SYNDROME?

• National Human Genome Research Institute
• EyeWiki

Updated 04/2023

#Conditions

Print Version

Print Version (In Arabic)


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.

What is the cause of Duane Syndrome?

Duane syndrome is due to miswiring of nerves to the eye muscles.

In Duane syndrome, the 6th cranial nerve that controls the lateral rectus muscle (the muscle that rotates the eye out towards the ear) does not develop properly. Why the nerve does not develop is not fully understood. Thus, the problem is not primarily with the eye muscle itself, but with the nerve that controls to the muscle. There can also be associated with miswiring of the 3rd cranial nerve, which normally controls the medial rectus muscle (the muscle that rotates the eye toward the nose). This is why abnormalities may be found in both left gaze and right gaze.

Who gets Duane Syndrome?

The vast majority of cases occur spontaneously and most often only affect one eye. Around 20% of Duane syndrome patients have both eyes affected. For reasons unknown, the left eye is more often involved than the right eye. Duane syndrome affects girls more often than boys. No particular race or ethnic group is more likely to be affected. 30% of cases are associated with other congenital anomalies.

What are the characteristics of Duane Syndrome?

• Strabismus: the eyes may be misaligned and point in different directions some or all of the time

• Head position: patients often maintain an abnormal head posture or head turn to keep the eyes straight

• Amblyopia (reduced vision in the affected eye): occurs in 10% of patients

• Eyelid narrowing: the affected eye may appear smaller than the other eye

• Upshoot or downshoot: with certain eye movements, the eye may occasionally deviate upward or downward

Is Duane syndrome congenital (present from birth)?

Duane retraction syndrome is present from birth, even if it is not recognized during infancy. An abnormal head posture and strabismus are often visible in old photographs taken in early childhood.

Is Duane syndrome hereditary?

Most of the time, it is not hereditary. In 90% of cases, the patient has no family history of Duane syndrome. Only in 10% of patients will have an affected family member and these tend to be cases where both eyes are involved.

Are there different types of Duane Syndrome?

Duane syndrome is often characterized by whether the primary abnormality is a reduced ability to turn the affected eye(s) outward (type I), inward (type II), or both (type III). Type I is the most common form of Duane syndrome, and affected patients will characteristically have a head turn towards the involved side, and will appear esotropic (crossed-eye) in straight ahead gaze.

Do Duane Syndrome patients have other eye problems?

The problem with the 6th cranial nerve is usually an isolated condition and the child is usually otherwise completely normal. With careful follow-up, the long-term prognosis for good vision is usually excellent.

Occasionally, Duane syndrome may be found in association with other eye problems, including disorders of other cranial nerves, nystagmus (an involuntary back-and-forth movement of the eyeball), cataract, optic nerve abnormalities, microphthalmos (abnormally small eye), and crocodile tears.

Do Duane Syndrome patients have non-ocular medical problems?

Not usually, however, some patients with Duane syndrome have other problems, such as hearing impairment, Goldenhar syndrome, spinal and vertebral abnormalities. There is also an increased frequency of Duane syndrome in patients with thalidomide exposure.

When is Duane Syndrome treated?

For the majority of patients, Duane syndrome does not require surgical treatment. Surgery for Duane syndrome is indicated for one of four reasons:

1. To reduce a significant deviation in normal straight-ahead position

2. To eliminate a significant abnormal head position

3. To eliminate a significant upshoot or downshoot.

4. To eliminate disfiguring abnormal eyelid position

The goal of treatment is to restore satisfactory eye alignment in the straight-ahead position, eliminate an abnormal head posture and to prevent amblyopia. Eye muscle surgery is not always required. Because the function of the affected nerve and muscle cannot be restored, the other eye muscles are adjusted to compensate and allow for better eye alignment.

How successful is surgery for Duane Syndrome?

Surgery cannot actually fix the problem of nerves that are miswired, but can move muscles to compensate for the miswiring. Because surgery cannot fix the problem of the abnormal nerves, surgery cannot restore normal eye movement. However, surgery can (and usually does) substantially improve the situation. The full effect of the surgery may take some weeks to become apparent.

Where can I find more information regarding Duane syndrome?

• National Human Genome Research Institute
• EyeWiki

Updated 10/2020