Moebius syndrome

How is it produced?

The cranial nerves are the twelve pairs of nerves that originate at the base of the brain and exit from the openings at the base of the skull to enervate various areas of the head. The sixth cranial or external ocular motor is in charge of mobilizing the eyeball outwards, while the seventh cranial or facial nerve is the one in charge of the mobility of the facial muscles, that is, it is the nerve that allows us to gesticulate. with the face.

The exact origin of Moebius syndrome is not known. It is considered that there may be a lack of formation of the nerve nuclei where these nerves originate bilaterally, or that during embryonic development there is a failure in the irrigation of these areas and the nuclei in formation of said nerves atrophy.

Likewise, it has also been seen that children with this syndrome are often the children of mothers who suffered profuse bleeding during pregnancy, a fact that can lead to a marked and, therefore, a decrease in fetal blood flow. Other placental alterations or the use of vasoconstrictor drugs during pregnancy have also been associated with this syndrome. It is not a hereditary disease.

Symptoms

The alteration of the seventh cranial nerve causes the patient not to have facial expressiveness. In the newborn it is very evident because the child cries and produces tears but with little alteration of his face, since he cannot wrinkle his forehead, close his eyes forcefully or gesture with his mouth, also preventing them from smiling. The affectation is more marked in the upper half of the face, in the forehead, eyebrows and eyes.

These are children who do not suck well because they cannot move their lips to cling to the nipple or the nipple of the bottle, so they tend to reject food. In addition, poor mouth occlusion causes frequent and profuse drooling, as well as changes in dental arrangement and frequent cavities.

The involvement of the sixth cranial nerve means that the eyes cannot move outwards, in a way that children present. Furthermore, as they cannot blink correctly, they cannot close their eyes even when they sleep, although when they try to do so, their eyes rise upward, and the absence of blinking also predisposes them to corneal injury.

They may have speech disturbances due to not being able to correctly emit certain sounds, especially labials and fricatives, [b], [p], [m] and [f]. The difficulty in speaking, as well as his lack of expressiveness, are a drag on the adequate psychosocial development of the child. Despite all this, there is not the least degree of mental retardation, they are people with fully preserved intellectual capacities.

Often there are also alterations of other cranial nerves, such as the eighth (which affects hearing and balance), the tenth (which affects the palate) or the twelfth (which affects the tongue). Sometimes malformations of the jaw or tongue, cleft palate, malformation of the pectoral muscles, club feet or absence of fingers or toes are manifested jointly.

Diagnosis

The diagnosis is based on the observation of the patient, observing both parents and pediatricians the strabismus and the lack of expression of the newborn. In principle, other complementary tests are not necessary, but sometimes a facial (EMG) is performed.

Treatment

The initial treatment consists of achieving a correct feeding of the baby to ensure proper development. Likewise, it is advisable to use artificial tears to avoid corneal ulcers.

Speech therapists can help children express themselves better despite their difficulty moving their mouths and making certain sounds.

Poor lid closure and strabismus can be surgically corrected, as well as other associated malformations that the patient may present. Nowadays, some interventions have been successfully carried out at an early age that have made it possible to improve facial mimicry to ensure that the patient can smile, but at the moment there are not too many cases treated in this way.

Precautionary measures

There are no preventive measures for this disease. In case of suspecting it in a newborn, contact your pediatrician as soon as possible.

• The alteration of the seventh cranial nerve causes the patient to have no facial expressiveness, especially in the upper half of the face, in the forehead, eyebrows and eyes.
• Consequences: poor mouth occlusion that causes them to reject food or drool frequently, difficulty in blinking correctly or making certain sounds ...
• Difficulty in speech and lack of expressiveness are a drag on the proper psychosocial development of the child, but there is not the least degree of mental retardation.