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Duane syndrome

Duane syndrome (DS) is a rare, congenital eye movement disorder most commonly characterized by the inability of the eye to turn out. Most patients are diagnosed by the age of 10 years and DS is more common in girls (60 percent of the cases) than boys (40 percent of the cases). The syndrome was named for Alexander Duane who identified it in 1905. more...

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DS is 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. People with DS have a limited (and sometimes absent) ability to move the eye outward toward the ear (abduction) and, in most cases, a limited ability to move the eye inward toward the nose (adduction).

Often, when the eye moves toward the nose, the eyeball also pulls into the socket (retraction), the eye opening narrows and, in some cases, the eye will move upward or downward. Many patients with DS develop a face turn to maintain binocular vision and compensate for improper turning of the eyes.

In about 80 percent of cases of DS, only one eye is affected, most often the left. However, in some cases, both eyes are affected, with one eye usually more affected than the other.

Other names for this condition include: Duane's Retraction Syndrome (or DR syndrome), Eye Retraction Syndrome, Retraction Syndrome, Congenital retraction syndrome and Stilling-Turk-Duane Syndrome.

Read more at Wikipedia.org


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Eye muscle surgery
From Gale Encyclopedia of Medicine, 4/6/01 by Lorraine Lica

Definition

Eye muscle surgery is surgery to weaken, strengthen, or reposition any of the muscles that move the eyeball (the extraocular muscles).

Purpose

The purpose of eye muscle surgery is generally to align the pair of eyes so that they gaze in the same direction and move together as a team, either to improve appearance or to aid in the development of binocular vision in a young child. To achieve binocular vision, the goal is to align the eyes so that the location of the image on the retina of one eye corresponds to the location of the image on the retina of the other eye.

In addition, sometimes eye muscle surgery can help people with other eye disorders (nystagmus and Duane syndrome, for example).

Precautions

Depth perception (stereopsis) develops around the age of three months old. For successful development of binocular vision and the ability to perceive three-dimensionally, the surgery should not be postponed past the age of four. The earlier the surgery the better the outcome, so an early diagnosis is important. Surgery may even be performed before two years old. After surgery, if binocular vision is to develop, corrective lenses and eye exercises (vision therapy) will probably be necessary.

Description

The extraocular muscles attach via tendons to the sclera (the white, opaque, outer protective covering of the eyeball) at different places just behind an imaginary equator circling the top, bottom, left, and right of the eye. The other end of each of these muscles attaches to a part of the orbit (the eye socket in the skull). These muscles enable the eyes to move up, down, to one side or the other, or any angle in between.

Normally both eyes move together, receive the same image on corresponding locations on both retinas, and the brain fuses these images into one three-dimensional image. The exception is in strabismus which is a disorder where one or both eyes deviate out of alignment, most often outwardly (exotropia) or toward the nose (esotropia). The brain now receives two different images, and either suppresses one or the person sees double (diplopia). This deviation can be adjusted by weakening or strengthening the appropriate muscles to move the eyes toward the center. For example, if an eye turns upward, the muscle at the bottom of the eye could be strengthened.

Rarely, eye muscle surgery is performed on people with nystagmus or Duane syndrome. Nystagmus is a condition where one or both eyes move rapidly or oscillate; it can sometimes be helped by moving the eyes to the position of least oscillation. Duane syndrome is a disorder where there is limited horizontal eye movement; it can sometimes be relieved by surgery to weaken an eye muscle.

There are two methods to alter extraocular muscles. Traditional surgery can be used to strengthen, weaken, or reposition an extraocular muscle. The surgeon first makes an incision in the conjunctiva (the clear membrane covering the sclera), then puts a suture into the muscle to prevent it from getting lost and loosens the muscle from the eyeball with a surgical hook. During a resection, the muscle is detached from the sclera, a piece of muscle is removed so the muscle is now shorter, and the muscle is reattached to the same place. This strengths the muscle. In a recession, the muscle is made weaker by repositioning it. More than one extraocular eye muscle might be operated on at the same time.

Another way of weakening eye muscles, using botulinum toxin injected into the muscle, was introduced in the early 1980s. Although the botulinum toxin wears off, the realignment may be permanent, depending upon whether neurological connections for binocular vision were established during the time the toxin was active. This technique can also be used to adjust a muscle after traditional surgery.

The cost of eye muscle surgery is about $2,000-$4,000, and about 700,000 surgeries are performed annually in the United States.

Preparation

Patients should make sure their doctors are aware of any medications that they are taking, even over-the-counter medications. Patients should not take aspirin, or any other blood-thinning medications for ten days prior to surgery, and should not eat or drink after midnight the night before.

Aftercare

Patients will need someone to drive them home after their surgery. They should continue to avoid aspirin and other non-steroidal anti-inflammatory agents for an additional three days, but they can take acetaminophen (e.g., Tylenol). Patients should discuss this with the surgeon to be clear what medications they can or cannot take. Pain will subside after two to three days, and patients can resume most normal activities within a few days. Again, this may vary with the patient and the patient should discuss returning to normal activity with the surgeon. They should not get their eyes wet for three to four days and should refrain from swimming for ten days. Operated eyes will be red for about two weeks.

Risks

As with any surgery, there are risks involved. Eye muscle surgery is relatively safe, but very rarely a cut muscle gets lost and can not be retrieved. This, and other serious reactions, including those caused by anesthetics, can result in vision loss in the affected eye. Occasionally, retinal or nerve damage occurs. Double vision is not uncommon after eye muscle surgery. As mentioned earlier, glasses or vision therapy may be necessary.

Normal results

Cosmetic improvement is likely with success rate estimates varying from about 65-85%. According to the best statistics as of 1998, binocular vision is improved in young children about 35% of the time. There is no improvement, or the condition worsens 15-35% of the time. A second operation may rectify less-than-perfect outcomes.

Key Terms

Botulinum toxin (botulin)
A neurotoxin made by ; causes paralysis in high doses, but is used medically in small, localized doses to treat disorders associated with involuntary muscle contraction and spasms, in addition to strabismus.
Conjunctiva
The mucous membrane that covers the eyes and lines the eyelids.
Extraocular muscles
The muscles (lateral rectus, medial rectus, inferior rectus, superior rectus, superior oblique, and inferior oblique) that move the eyeball.
Orbit
The cavity in the skull containing the eyeball; formed from seven bones: frontal, maxillary, sphenoid, lacrimal, zygomatic, ethmoid, and palatine.

Retina
The inner, light-sensitive layer of the eye containing rods and cones; transforms the image it receives into electrical messages sent to the brain via the optic nerve.
Sclera
The tough, fibrous, white outer protective covering of the eyeball.
Strabismus
A disorder where the two eyes do not point in the same direction.

Further Reading

For Your Information

    Books

  • Good, William V., and Craig S. Hoyt. Strabismus Management. Boston: Butterworth-Hienemann, 1996.
  • Salmans, Sandra. Your Eyes: Questions You Have...Answers You Need. Allentown, PA: People's Medical Society, 1996.
  • von Noorden, Gunter K. Binocular Vision and Ocular Motility: Theory and Management of Strabismus, 5th ed. St. Louis, MO:Mosby-Year Book, 1996.

    Organizations

  • American Academy of Ophthalmology. P.O. Box 7424, San Francisco, CA 94120-7424. (415) 561-8500. http://www.eyenet.org/.
  • American Association for Pediatric Ophthalmology and Strabismus (AAPOS). PO Box 193832, San Francisco, CA 94119-3832. (415) 561-8505. http://med-aapos.bu.edu/.

    Other

  • Olitsky, Scott E., and Leonard B. Nelson. Strabismus Web Book http://www.smbs.buffalo.edu/oph/ped/webbook.htm. (4 May 1998).

Gale Encyclopedia of Medicine. Gale Research, 1999.

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