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Duane syndrome (DS) is a rare, congenital (present from birth) eye movement disorder. 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).
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). 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. Eden was diagnosed with Type 1 DS this past February. It seems to mainly affect her right eye. She has difficulty moving her right eye outwards. We are still learning about DS; causes, treatments (if any), and how it will affect her later in life. This blog aims to provide information about DS as I learn about it, also to provide encouragement for other families affected by this syndrome.
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Sunday, 12 April 2009 17:26 |
Disease characteristics. Duane syndrome is a strabismus syndrome characterized by congenital non-progressive horizontal ophthalmoplegia (inability to move the eyes) primarily affecting the abducens nucleus and nerve and its innervated extraocular muscle, the lateral rectus muscle. At birth, affected individuals have restricted ability to move the affected eye(s) outward (abduction) and/or inward (adduction). In addition, the globe retracts into the orbit with attempted adduction, accompanied by narrowing of the palpebral fissure. Most individuals with Duane syndrome have strabismus in primary gaze but can use a compensatory head to align the eyes, and thus can preserve single binocular vision and avoid diplopia. Individuals with Duane syndrome who lack binocular vision are at risk for amblyopia. Approximately 70% of individuals with Duane syndrome have isolated Duane syndrome; i.e., they do not have other detected congenital anomalies. Read more about the managment of Duane Syndrome, genetic counseling...etc. Link |
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Sunday, 22 March 2009 11:53 |
The treatment of Duane syndrome is surgical. The surgery is aimed at reducing the unwanted effects of co-contraction of the medial and lateral rectus muscles. Successful surgery results in a straighter head position, a lessening of enophthalmos and upshoot and downshoot, and better alignment in primary position. Attempts at improving abduction are less effective and may even be harmful if the lateral rectus is overly tightened because this increases enophthalmos.
The findings typical of Duane syndrome can be observed even in the very young, at a few months of age. Infants are often evaluated to rule out sixth nerve palsy from another cause or simply for esotropia. Surgery can be done at any time, but is usually deferred until the child is walking. Some reasons to consider surgical treatment early are concerns over motor development (walking, catching a ball, etc.), increased stiffness of the lateral rectus, and problems associated with the face turn (both appearance and posture). Link |
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