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Duane syndrome—which is also known as Duane’s syndrome or Duane retraction syndrome— is a rare form of strabismus, or misalignment of the eye. Where most forms of strabismus leave side-to-side eye movements intact, in Duane syndrome the “wiring” of the eye muscles gets jumbled, and movement of eye(s) is limited in certain directions.. The disorder is named after Alexander Duane, an early-20th-century ophthalmologist who devoted his career to helping children with the condition.
What goes wrong in the eye of a person who has Duane syndrome?
The problem in Duane syndrome is actually the nerve controlling the eye muscle—not the muscles themselves.
To better understand Duane syndrome, it’s helpful to understand the basic anatomy of the eye.
When a person has Type I Duane syndrome:
What are the different types of Duane syndrome?
There are three types of Duane syndrome. In all three types, the opening of the eye narrows and the eyeball retracts when the eye moves inward
By far the most common is Type I, in which, in addition to the retraction of the eye:
In Type II Duane syndrome:
In Type III Duane syndrome:
Because the majority of patients with Duane syndrome have Type I, we will focus on that form of the condition throughout these pages. Your doctor can give you more information about Type II or Type III Duane syndrome.
Who is more likely to develop Duane syndrome?
The disorder affects people from all races and ethnic backgrounds. For reasons we don’t yet understand, girls are more likely to develop Duane syndrome than boys.
Is Duane syndrome linked to any other conditions?
The majority of people with Duane syndrome do not have any other health problems. However, in rare cases, patients with the disorder also have complex conditions such as:
Is there a cure for Duane syndrome?
Because the affected sixth cranial nerve cannot be repaired or replaced, there is no cure for Duane syndrome. However, for people whose lives are significantly disrupted by the condition, surgery can be very helpful in:
Read more about surgical treatments for Duane syndrome.
Watch as ophthalmologist-in-chief, David G. Hunter, MD, PhD, and the Ophthalmology team at Children’s Hospital Boston address the issues of Duane syndrome in this surgical webcast.
What causes Duane syndrome?
Duane syndrome occurs when the sixth cranial nerve—the nerve originating in the brainstem that controls the movements of the lateral rectus muscle—either doesn’t develop at all, or doesn’t work properly. We are working to uncover an exact reason for this faulty development, and the laboratory of Children's researcher Elizabeth Engle, MD, has discovered genes that cause some of the more rare forms of the condition.
If there is no nerve to carry the signal from the brain to the muscle:
But with this jumbled wiring:
In some people, the lateral rectus muscle overcomes the medial rectus muscle in the tug of war, and the eye can move outward but not inward (Duane syndrome Type II). In other cases, neither the lateral nor the medial rectus muscle dominates, and the eye does not move well either inward or outward (Duane syndrome Type III.)
Is Duane syndrome congenital (present at birth)?
Yes. Children with Duane syndrome are born with the disorder. However, it might not become readily apparent until the child grows older and starts demonstrating difficulty with vision or an abnormal head turn to the side (see the Symptoms section below to learn more). Most cases of Duane syndrome are diagnosed by age 10.
Is Duane syndrome inherited?
In most cases, no. Ninety percent of children with Duane syndrome have no family history of the disorder. About 5 percent of people with Duane syndrome have a parent with Duane syndrome (dominant inheritance). Here at Children's, our research team has discovered the CHN1 mutation in patients with dominantly inherited Duane syndrome.
Is there a genetic test to screen for Duane syndrome?
No. There is currently no test available that can detect a genetic marker for the disorder, except for the CHN1 genetic test when Duane syndrome affects more than one generation in a family.
Could I have done anything to prevent Duane syndrome?
No. There is no scientific evidence that suggests Duane syndrome is caused by anything a parent or patient did, or didn’t, do.
What are the symptoms of Duane syndrome?
Although Duane syndrome is congenital (present at birth), it may take as long as several years for it to become noticeable: very young children cannot vocalize the fact that they are having trouble with their vision, while older children may have learned to compensate very well for their visual difficulties. In both cases, the symptoms children show can be quite subtle. Most people with Duane syndrome are diagnosed by age 10.
When symptoms are noticeable, they usually include:
Older children may be able to describe the problems they are experiencing, such as:
Since these symptoms can also be caused by other eye disorders, it’s important to obtain an evaluation from a qualified eye doctor as soon as possible.
Read one patient’s message of thanks for the Children’s Ophthalmology team.
Q: Is Duane syndrome common?
A: No; it is a rare disorder, affecting fewer than 200,000 people in the United States according to the Office of Rare Diseases of the National Institutes of Health.
Q: What’s the difference between strabismus and Duane syndrome?
A: Strabismus is any eye disorder that involves misalignment of the two eyes. Duane syndrome is one type of strabismus that is distinct because of the retraction of the eye.
Q: Does Duane syndrome cause blindness?
A: No. While one eye can lose vision (see next question), the condition does not cause blindness.
Q: If a person has Duane syndrome, will there be other eye problems, too?
A: Most likely, no. Some children with Duane syndrome will also develop amblyopia, or “lazy eye,” in which a normal eye loses vision because the brain is not paying attention to the input. This can develop in Duane syndrome if the head position that usually develops is not sufficient to compensate for the weak muscle and the eyes becomes constantly misaligned.
There are many clinical reports of patients with Duane syndrome who have other eye conditions. However there is no proof that these other eye conditions occur any more commonly in Duane syndrome than they do in other people.
Q: Is Duane syndrome linked to any other health conditions (beyond vision problems)?
A: The majority of children with Duane syndrome do not have any other health problems. However, in rare instances, children with the disorder may also have complex conditions, such as:
Q: Is it common for people with Duane syndrome to be shy or withdrawn around others?
A: In cases where the eye alignment is significantly disrupted, yes. It’s important to remember that a big part of human communication is looking people in the eye. A person with moderate to severe Duane syndrome might be unable to make and sustain good eye contact. Understandably, this can make her feel self-conscious and less able to connect with others.
Q: Are there certain activities a person with Duane syndrome should avoid?
A: This depends on how severely the eye alignment is affected by Duane syndrome. In general, a person who experiences double vision and/or poor depth perception should be extra careful when doing things like going up and down stairs, playing sports and crossing the street. Extra care may also be needed when looking toward the side of the affected eye. Your treating ophthalmologist can give you detailed recommendations.
Q: Is eye surgery definitely required for Duane syndrome?
A: Not necessarily. Many people with Duane syndrome don’t have any symptoms that interfere with their day-to-day lives. They are able to compensate for their lost or reduced eye movement with just a slight head turn. These people may need nothing more than long-term monitoring by their eye doctor.
People whose symptoms are more severe—for example, people with a serious degree of eye misalignment or with a pronounced head turn—may need eye muscle surgery. Your treating clinician will work with you to determine the best next steps for your particular circumstances.
Q: Can Duane syndrome be cured?
A: No. There is no surgery or other treatment that can fix the missing or improperly developed cranial nerve responsible for the limited motion in the eye muscle. However, doctors can use surgery to reposition the other eye muscles—allowing them to balance better and keep the eyes in better alignment.
Q: Are there any long-term health effects of having Duane syndrome?
A: The vast majority of people with Duane syndrome do just fine—many don’t even need surgery.
People whose Duane syndrome significantly impacts their daily lives—with symptoms like double vision, headaches and an abnormal head turn adopted in an attempt to see better—are at risk of long-term health issues without proper treatment. These problems can include:
In addition, because a more serious case of Duane syndrome can prevent initiation of normal eye contact, the right treatment is necessary to prevent problems in adolescence and adulthood, such as:
Ophthalmologists at Children’s have developed the Pediatric Vision Scanner (PVS), a device that, in a quick 2.5-second scan of the eyes, can detect strabismus, amblyopia and other serious eye conditions in children as young as 2. Read more.
You and your family play an essential role in the treatment of Duane syndrome. It’s important that you share your observations with the treating ophthalmologist, and that you have all the information you need to fully understand the treatment team’s explanations and recommendations.
It’s often very helpful to jot down your thoughts and questions ahead of time and bring them with you, along with a notebook, to your appointment. That way, you will have all of your questions in front of you when you meet with the treating clinician and can make notes to take home with you. Older children with Duane syndrome can be encouraged to write down questions, too.)
Initial questions to ask your doctor might include:
Children’s is one of just a handful of pediatric ophthalmology programs in the country with a service dedicated entirely to eye problems caused by neurological conditions. Learn more about our new Neuro-Ophthalmology Service.
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