Anyone with Duane syndrome, I have a baby with it,any info would be helpful.?!


Question: Good Morning,

I'm providing you an article written by a doctor that will explain this condition much more thoroughly to you. I hope it is beneficial to you.

Have a great day and read on....

Author: Arun Verma, MD, Senior Consultant, Department of Ophthalmology, Dr Daljit Singh Eye Hospital, India

Background

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 toward the ear 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 syndrome?

Duane syndrome (DS) is a rare, congenital disorder of eye movement. In most patients, DS is diagnosed by the age of 10 years.

DS is a miswiring of the eye muscles that causes some eye muscles to contract when they should not and other eye muscles not to contract when they should. People with DS have a limited and sometimes absent ability to move their eye outward toward the ear (ie, abduction), and in most cases, they have a limited ability to move the eye inward toward the nose (ie, adduction).

This probably occurs around the sixth week of pregnancy and is due to poor development of tiny parts of the brainstem that control the eye muscles. In DS, the sixth cranial nerve that controls the lateral rectus muscle (the muscle that rotates the eye out toward the ear) does not develop properly. Why the nerve does not develop is not yet understood. Thus, the problem is not primarily with the eye muscle itself but with the nerve that transmits the electrical impulses to the muscle. There is also irregular innervation of a branch from the third cranial nerve, which controls the medial rectus muscle (the muscle that rotates the eye toward the nose). This is why abnormalities may be found in both left gaze and right gaze.

Often, when the eye moves toward the nose, the eyeball also pulls into the socket (ie, retraction), and the eye opening narrows. In some cases, the eye moves upward or downward. Many patients with DS develop a habit of turning their face to maintain binocular vision and thus compensate for improper turning of the eyes. In about 80% of all cases of DS, only 1 eye is affected, most often the left eye. However, in some cases, both eyes are affected; usually, 1 eye is affected more than the other.

In 70% of patients, DS is the only disorder the individual has. However, other conditions and syndromes have been associated with DS. These include malformation of the skeleton, ears, eyes, kidneys, and nervous system, as well as the following: Okihiro syndrome (an association of DS with forearm malformation and hearing loss), Wildervanck syndrome (fusion of neck vertebrae and hearing loss), Holt-Oram syndrome (abnormalities of the upper limbs and heart), morning-glory syndrome (abnormalities of the optic disc or blind spot), and Goldenhar syndrome (malformation of the jaw, cheek, and ear, usually on 1 side of the face).

What is Duane retraction syndrome?

Sinclair (in 1895), Bahr (in 1896), Stilling (in 1887), Turk (in 1899), and Wolff (in 1900) first described Duane retraction syndrome (DRS).1, 2, 3, 4, 5 In 1905, Duane reported 54 cases, summarizing all the clinical findings, reviewing previous work, and offering theories on the pathogenesis and treatment of the disease.6

Brown classified the condition into 3 subtypes, based upon clinical observations.7 Type A corresponded to limited abduction and less limited adduction (as described originally by Duane). Type B corresponded to limited abduction but normal adduction. Type C corresponded to limitation of adduction that is greater than limitation of abduction, giving rise to a divergent deviation and a head posture in which the face is turned away from the side of the affected eye.

In 1974, with the support of electromyography (EMG) and following the suggestions of Lyle and Malbran, Huber classified DRS into the 3 types: Duane 1, Duane 2, and Duane 3.8, 9, 10 Type 1 is marked limitation of abduction (corresponds to Type B in Brown's classification) explicable by maximum innervation reaching the lateral rectus muscle only when the affected eye is adducted. Type 2 is limitation of adduction (corresponds to Type C in Brown
Answers: Good Morning,

I'm providing you an article written by a doctor that will explain this condition much more thoroughly to you. I hope it is beneficial to you.

Have a great day and read on....

Author: Arun Verma, MD, Senior Consultant, Department of Ophthalmology, Dr Daljit Singh Eye Hospital, India

Background

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 toward the ear 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 syndrome?

Duane syndrome (DS) is a rare, congenital disorder of eye movement. In most patients, DS is diagnosed by the age of 10 years.

DS is a miswiring of the eye muscles that causes some eye muscles to contract when they should not and other eye muscles not to contract when they should. People with DS have a limited and sometimes absent ability to move their eye outward toward the ear (ie, abduction), and in most cases, they have a limited ability to move the eye inward toward the nose (ie, adduction).

This probably occurs around the sixth week of pregnancy and is due to poor development of tiny parts of the brainstem that control the eye muscles. In DS, the sixth cranial nerve that controls the lateral rectus muscle (the muscle that rotates the eye out toward the ear) does not develop properly. Why the nerve does not develop is not yet understood. Thus, the problem is not primarily with the eye muscle itself but with the nerve that transmits the electrical impulses to the muscle. There is also irregular innervation of a branch from the third cranial nerve, which controls the medial rectus muscle (the muscle that rotates the eye toward the nose). This is why abnormalities may be found in both left gaze and right gaze.

Often, when the eye moves toward the nose, the eyeball also pulls into the socket (ie, retraction), and the eye opening narrows. In some cases, the eye moves upward or downward. Many patients with DS develop a habit of turning their face to maintain binocular vision and thus compensate for improper turning of the eyes. In about 80% of all cases of DS, only 1 eye is affected, most often the left eye. However, in some cases, both eyes are affected; usually, 1 eye is affected more than the other.

In 70% of patients, DS is the only disorder the individual has. However, other conditions and syndromes have been associated with DS. These include malformation of the skeleton, ears, eyes, kidneys, and nervous system, as well as the following: Okihiro syndrome (an association of DS with forearm malformation and hearing loss), Wildervanck syndrome (fusion of neck vertebrae and hearing loss), Holt-Oram syndrome (abnormalities of the upper limbs and heart), morning-glory syndrome (abnormalities of the optic disc or blind spot), and Goldenhar syndrome (malformation of the jaw, cheek, and ear, usually on 1 side of the face).

What is Duane retraction syndrome?

Sinclair (in 1895), Bahr (in 1896), Stilling (in 1887), Turk (in 1899), and Wolff (in 1900) first described Duane retraction syndrome (DRS).1, 2, 3, 4, 5 In 1905, Duane reported 54 cases, summarizing all the clinical findings, reviewing previous work, and offering theories on the pathogenesis and treatment of the disease.6

Brown classified the condition into 3 subtypes, based upon clinical observations.7 Type A corresponded to limited abduction and less limited adduction (as described originally by Duane). Type B corresponded to limited abduction but normal adduction. Type C corresponded to limitation of adduction that is greater than limitation of abduction, giving rise to a divergent deviation and a head posture in which the face is turned away from the side of the affected eye.

In 1974, with the support of electromyography (EMG) and following the suggestions of Lyle and Malbran, Huber classified DRS into the 3 types: Duane 1, Duane 2, and Duane 3.8, 9, 10 Type 1 is marked limitation of abduction (corresponds to Type B in Brown's classification) explicable by maximum innervation reaching the lateral rectus muscle only when the affected eye is adducted. Type 2 is limitation of adduction (corresponds to Type C in Brown



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