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Duane Syndrome in Children

  • Overview

    The Boston Children's Hospital team approaches each case of Duane syndrome with a patient-focused, family-centered approach, drawing from the extensive multidisciplinary expertise within our Department of Ophthalmology and across the hospital.

    If you or your child have been diagnosed with an eye disorder like Duane syndrome, you may have dozens of questions: What does this condition entail? How will it affect the future? Will surgery be required? Does the condition affect vision? What do we do next?

    Here at Children’s Hospital Boston, our experts in strabismus and pediatric ophthalmology have decades of experience treating Duane syndrome (and the full spectrum of related vision disorders). The information on the following pages is designed to give you a helpful overview of the condition, as well as a sense of the available treatment options.

    Rest assured that our compassionate, knowledgeable clinicians are here to support you and your family at every step of the way. 

    What is Duane syndrome, exactly?
    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. There are three types of Duane syndrome, but the most common is Type I, which interferes with the eye’s ability to move outward (away from the nose). Duane syndrome can usually affects just one eye, but it can sometimes involve both.  The left eye is more likely to be affected than the right, although doctors still don’t know exactly why.

    Learn more about the other types.

    To better understand Duane syndrome, it’s helpful to understand the basic anatomy of the eye

    • Three of the nerves extending from the brain to the eye muscles, called cranial nerves, control the movements of the eye by transmitting electrical signals.
    • The sixth cranial nerve is in charge of the lateral rectus muscle, the muscle that moves the eye outward.
    • The third cranial nerve controls the medial rectus muscle, the muscle that pulls the eye inward, as well as other muscles.

     When a person has Type I Duane syndrome:

    • The lateral rectus muscle does not receive a signal from the sixth cranial nerve.
    • The eye is unable to move fully outward (away from the nose).
    • Instead, the lateral rectus muscle receives a signal from the third cranial nerve.
    • When the eye moves inward, the lateral rectus muscle activates, and the eye appears to shrink in size (retract) as both the medial and lateral rectus muscles pull at the same time. This is why Duane syndrome is sometimes called “Duane retraction syndrome.” 

    Sometimes, Duane syndrome occurs because the sixth cranial nerve does not develop at all; in other cases, the nerve develops improperly or doesn’t function correctly. The image at left, courtesy of the research webpage of Elizabeth Engle, MD, shows the missing pathway of the sixth cranial nerve and its relation to the third cranial nerve. Click for a larger version of the image.

    How Children’s Hospital Boston approaches Duane syndrome
    Children's doctors are known nationwide for handling the most complex cases of Duane syndrome and related disorders. Our ophthalmologic surgeons specialize in the delicate eye muscle operations that are required to treat the syndrome, including adjustable suture surgery and vertical transposition surgery

    Children’s ophthalmologists are actively involved in research that is shaping the way eye disorders like Duane syndrome are detected and treated around the world. And, since Children’s is Harvard Medical School’s primary teaching hospital for pediatrics and pediatric ophthalmology, our doctors are also training the next generation of clinicians and clinical scientists. 

    Our team treats each person with Duane syndrome with a patient-focused, family-centered approach, drawing from the extensive multidisciplinary expertise within our Department of Ophthalmology and across the hospital.

    Although we are known for our science-driven approach to medicine at Children’s, we always remember that every patient is, first and foremost, an individual. Our team of professionals will create a customized treatment plan for specific symptoms and circumstances—and we’ll include you and your family at every step of the way. 

    Spotlight on: Children's Pediatric and Adult Strabismus Services
    The Pediatric and Adult Strabismus Services at Children's offer comprehensive evaluation and correction of strabismus in babies, children and adults. Adults with strabismus are referred to Children’s because our ophthalmologists, who are specially trained in childhood eye conditions, have years of expertise in the delicate eye muscle surgery typically required to straighten the eyes.

    Duane syndrome: Reviewed by David G. Hunter, MD, PhD
    © Children’s Hospital Boston; posted in 2011

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