Möbius syndrome is a rare congenital disorder of variable severity, involving multiple cranial nerves and predominantly characterized by bilateral or unilateral paralysis of the facial and abducens nerves. Paralysis of the VI and VII cranial nerves leads to a lack of function in the muscles they supply. Facial paralysis often causes bilabial incompetence with speech difficulties, oral incompetence, problems with eating and drinking, including storing food in the cheek and dribbling, as well as severe drooling (Bianchi, Copelli, Ferrari, Ferri, & Sesenna, 2009).
It is basically diagnosed by the physical features that the patient presents: bilateral facial paralysis, inexpressive fascie, “tent” mouth, mandibular hypoplasia, nail hypoplasia, clubfoot, limb defects and difficulty sucking and swallowing. Its etiology is multifactorial, although it has been associated with prenatal exposure to misoprostol (Mora, Chispe, & Prott, 2011). pointed out the association of diplegia with other malformations such as: multiple cranial neuropathies, mental retardation, endocrinological alterations, musculoskeletal deformity and cardiovascular defects. The incidence of Möbius syndrome is 1:50,000 to 100,000 (Pastuszak et al., 1998).
Causes
There are two forms of presentation: sporadic and familial. In the first case, transient hypoxic ischemic injury to the fetus due to alterations in maternal-fetal circulation has been suggested as the cause. In familial cases, forms of autosomal dominant (A/D), autosomal recessive (A/R) and X-linked transmission are recognized. Cytogenetic analysis of patients with Möbius Syndrome usually shows karyotypes.
normal constitutional laws.
The causes of this syndrome are unknown, however according to one of the most accepted theories, it would be atrophy of the cranial nuclei. The second theory suggests that the destruction or damage of the cranial nerve nuclei is due either to a lack of blood supply, or as a result of external effects such as infection, drugs or medications. Prenatal exposure to during the first trimester of pregnancy has been associated with Möbius syndrome, limb malformations, and vascular disruption has been proposed as the mechanism of teratogenic action.
According to the approach proposed by Roig Quilis, this syndrome is a brainstem dysgenesis and can be classified as: genetically determined, either in isolation or as part of a more extensive polymalformative syndrome, and acquired, due to prenatal destructive and disruptive lesions of the brain. vascular nature (Mora et al., 2011).
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Characterization
Paralysis of the orbicularis oculi muscle is the first sign of Möbius syndrome and can be observed shortly after birth; However, the condition is not well known within the pediatric specialty, so the diagnosis is made at older ages. As the child develops further, it becomes evident that the child does not smile or display other characteristics of non-verbal communication. Mask-like faces are the dominant clinical findings associated with drooling and slurred speech (Terzis & Noah, 2002).
Treatments
There is no medical treatment or cure for Möbius syndrome. It is managed with support and reduction of symptoms, and must be approached multidisciplinary, with the participation of different health professionals, such as pediatricians, speech therapists, maxillofacial surgeons, pediatric surgeons, children’s dentists, orthodontists, psychologists, among others (Morales- Chávez, Ortiz-Rincones, & Suárez-Gorrin, 2013).
Current treatments focus mainly on physical malformations. From this starting point, studies have been carried out on dental treatment (De Serpa Pinto, De Magalhães, & Nunes, 2002), as well as surgical treatment (microsurgical reconstruction for restoration of facial movements) (Terzis & Noah, 2002), among others. Surgical treatments aim to restore facial expression, achieve symmetry and facilitate patients’ smile. This brings great benefits to oral health, such as a good chewing process and support in the development of the jaws, if the surgery is performed during the patient’s growth period. It also facilitates the brushing process and makes the work of the health specialist easier when performing certain treatments in the dental office. This is due to better closure of the lips and a decrease in the chances of developing periodontal disease or rampant cavities. It also helps avoid mouth breathing, pronunciation difficulties, and swallowing problems (Morales-Chávez et al., 2013).
Processing of emotions and interpersonal relationships
According to a study on congenital facial paralysis and emotion processing (De Stefani, Nicolini, Belluardo, & Ferrari, 2019), the ability to detect and recognize facial expressions may confer adaptive advantages, such that a critical social signal can be decoded more efficiently. and quick to prepare an adaptive response. Studies on individuals with Möbius Syndrome, who have impairments in expressing emotions through the face from birth, reported that their inability to form facial expressions can affect their social interactions (Briegel, 2007, (Strobel & Renner, 2016)). and, therefore, their quality of life. Maintaining satisfactory peer relationships appears to be a special challenge for children and adolescents with Möbius syndrome (Strobel & Renner, 2016).
Furthermore, researchers are investigating whether adults with Möbius syndrome experience greater anxiety and depression or decreased social competence and life satisfaction compared to people without facial movement disorders. As a result, they found that people with Möbius syndrome reported significantly lower social competence than the control group, but did not differ significantly from the control or norm group in anxiety, depression, or life satisfaction. In people with Möbius syndrome, the degree of facial expression impairment was not significantly related to the adjustment variables. And they concluded that many people with Möbius syndrome are better adjusted than other research suggests, despite their difficulties with social interaction. To improve interaction, people with Möbius syndrome could compensate for the lack of facial expression with alternative expressive channels (Bogart & Matsumoto, 2010).
Another focus group explored the experiences and social interaction strategies of 12 adults with Möbius syndrome. Content analysis revealed five themes of social functioning: social engagement/disengagement; resilience/sensitivity; social support/stigma; be understood/misunderstood; and public awareness/lack of awareness about Möbius syndrome. Participants used compensatory expressive strategies such as vocal tone, gestures, and humor. The combination of not being able to express oneself with your face, having a facial difference and having a rare disease is particularly stigmatizing. Increasing public awareness and developing social skills programs for people with facial paralysis could facilitate social functioning (Briegel, 2006).
- Bianchi, B., Copelli, C., Ferrari, S., Ferri, A., & Sesenna, E. (2009). Facial animation in children with Moebius and Moebius-like syndromes. Journal of Pediatric Surgery, 44(11), 2236-2242. https://doi.org/
- Bogart, K.R., & Matsumoto, D. (2010). Living with Moebius syndrome: adjustment, social competence, and satisfaction with life. The Cleft Palate-Craniofacial Journal: Official Publication of the American Cleft Palate-Craniofacial Association, 47(2), 134-142. https://doi.org/
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- Morales-Chávez, M., Ortiz-Rincones, MA, & Suárez-Gorrin, F. (2013). Surgical techniques for smile restoration in patients with Möbius syndrome. Journal of Clinical and Experimental Dentistry, 5(4), e203-e207. https://doi.org/
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