Duane Retraction Syndrome

Duane's Syndrome - Type Of Squint

A: Child has a head turn where the face is slightly turned to her left

B: When the child looks right there is a slight narrowing of her left eye

C: When the child looks straight the left eye appears slightly inwards or Esotropia

D: When she looks to her left the left eye looks bigger and the left eye is not able to move beyond the midline

How do Normal Eye Movements occur?

The 6 muscles that control the movement of the eye are attached to the outside of the wall of the eye. In each eye, there are 2 muscles that move the eye horizontally. The lateral rectus muscle pulls the eye out and the medial rectus muscle pulls the eye in toward the nose. Four other muscles move the eye up or down and at an angle. Each eye muscle receives the command for movement from cranial nerves that exit the brain.

What is Duane's Syndrome?

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

Duane syndrome is due to the miswiring of the eye muscles. This causes some eye muscles to contract when they should not and other eye muscles not to contract when they should. This probably occurs around the sixth week of pregnancy and is due to the poor development of tiny parts of the brainstem that control the eye muscles. In Duane’s, the problem is not primarily with the eye muscle itself but with the nerve that transmits the electrical impulses to the muscle.

Duane syndrome is often characterized by whether the primary abnormality is a reduced ability to turn the affected eye(s) outward (Eso Duane's), inward (Exo Duane's), or both (type III).

What are the characteristics of Duane Syndrome?

• Strabismus-the eyes may be misaligned and point in different directions some or all of the time.
• Refractive Errors
• Head position-patients often maintain a head posture or head turn to keep the eyes straight.  In fact when we see children with Duane's syndrome and are following these children we instruct the parents to look out for the head posture.  If the head posture suddenly disappears it's not a good sign because then it means that the child may not be using both eyes and the child may thus develop Amblyopia.
• 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 down shoot-with certain eye movements, the eye may occasionally deviate upward or downward

Treatment

Treatment in dunes is usually done for one or more of the following reasons

• For head posture
• For a squint
• For improving the cosmetic appearance of the face ( the eyelid narrowing )
• The treatment involves operating on the eye muscles and changing their position so that the squint and head posture reduces.
• Depending on the type of dunes syndrome your surgeon will decide which muscles to operate upon.