Juliet Cohen

Detailed Information on Moebius syndrome

Moebius syndrome is thought to be genetic, and most cases are sporadic, occurring once in a family. The recurrence risk is thought to be extremely low, and one source has quoted it to be 2%.

Moebius Syndrome is an extremely rare condition which typically affects the sixth and seventh cranial nerves. These nerves affect the face and eye muscles.  Other cranial nerves may be affected, especially the 3rd, 4th, 5th, 9th, 10th and 12th. There may be skeletal involvement causing hand/feet anomalies and/or club feet.  Limb and chest wall abnormalities sometimes occur with the syndrome. Most people with Möbius syndrome are born with complete facial paralysis, which means they cannot close their eyes or form facial expression. The first symptom of Moebius syndrome, baby may also have difficulty sucking.

Other symptoms can involve: feeding, swallowing, and choking troubles; extreme drooling; crossed eyes; lack of facial expression; lack of ability to smile; eye sensitivity; motor delays; high or cleft palate; hearing problems; and speech difficulties. Small or absent brain stem nuclei that control the cranial nerves, as well as decreased numbers of muscle fibers, have been reported. Most people with Möbius syndrome have normal intelligence, and others should take care not to confuse their lack of facial expression with dullness or unfriendliness.

Moebius syndrome does not get worse over time. Treatment is based on the symptoms. Infants may need feeding tubes or special bottles to maintain sufficient nutrition if they have difficulty nursing. Physical, occupational, and speech therapy can improve motor skills and coordination, and lead to better control of speaking and eating abilities. Treatment for corneal ulcerations or abrasions may be required. This is secondary to exposure keratitis and conjunctivitis secondary to incomplete eyelid closure.

Limb and jaw deformities may frequently be improved through surgery. Tracheotomy may be essential to support the airway and permit tracheobronchial clearing. It is sometimes possible through surgery to counteract the facial paralysis by transferring nerves and muscles to the corners of the mouth. Additionally, the surgery cannot be considered a cure for Möbius syndrome since it does not improve the ability to form other facial expressions. In addition, plastic reconstructive surgery of the face can offer benefits in individual cases. Nerve and muscle transfers to the corners of the mouth have been performed to provide limited ability to smile.

Article Tags: Moebius Syndrome, Möbius Syndrome

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