Moebius Syndrome

Moebius syndrome is a rare, congenital (congenital) and non-transactive neurological syndrome, first described by German neurologist Paul Julius Moebius. Clinically, at least both VI. (abdusens) and VII. (facial) are characterized by the involvement of the correal nerve. It is often manifested by facial paralysis and constraints in eye movements at an outside view.^【1】 

The syndrome can be observed with wide phenotype variations. Basic findings:

• Mask-like facial expression
• Constraint in eye movements (horizontal ophthalmoplegia)
• Swallowing, absorption challenges
• Saliva flow in the mouth
• Atrophy in the language
• Ear, palate, limb and sternum anomalies (for example Poland syndrome)
• Intellectual retardation and/or behavioral disorders

Ophthalmological manifestations constitute an important aspect of Moebius syndrome. Particularly esotropia (introversion of the eye), exotropia (slip of the left) and the limitation in eye movements is common. Treatment is performed by strabismus surgeries, the use of glasses and the surface reconstruction if necessary. For example:

• Medial rectus muscle resection
• Inferior oblicit tenotomy
• Amnision membrane transplantation.

Before surgery, orbita and cranial MRI are recommended, since muscle hypoplasias (for example, the absence of lateral rectus muscle) can be seen.

In patients with educbius syndrome, there is a high risk of difficult intubation during anesthesia applications, especially due to oropy-diarrhea anomalies. Therefore, detailed evaluation before anesthesia is a must:

• Mallampati classification can be III or higher
• Equipment such as laryngolia mask, tracheostomy team should be kept ready
• Careful selection of premedication is important

In case presentations, anesthesia induction is usually provided with lovable gases, muscle is relaxed with vekuronium and careful intubation techniques are applied.^【2】

Studies in recent years show that autism spectrum disorders are more common in patients with Moebius syndrome. Among the reasons for this are:

• Common neurodevelopmental effects
• Brain stem anomalies
• Hypoxia, which occurs as a result of prenatal exposure to drugs such as misorostol

Diagnosis of autism can be difficult in these patients because facial gestures are weak and there is difficulty in making eye contact. Evaluation should be carried out using tests such as CARS, ABC and ADI-R. In some studies, the frequency of autism in patients with Moebius syndrome reached up to 26 %.^【3】 

Although the cause of moubius syndrome is not fully known, the two main hypotheses stand out:

1. Vascular Theory: Ischemic damage of brain stem nucleus with insufficiency of vertebral arteries in the 4th week of pregnancy
2. Genetic Theory: Genetic defects in brain stem development (such asHOXA-1 gene)

Exposure to drugs such as mioprostol and talidomide is defined as a risk factor in the development of this syndrome.

Moubus syndrome is a complex syndrome that requires a careful, multidisciplinary approach from a careful, neurological, ophthalmological, psychiatric and anesthetic. With early diagnosis, multidisciplinary follow-up, appropriate surgical and supportive therapies, the quality of life of the patients can be significantly improved.