A novel patient-controlled bidirectional palatal lift appliance.

OBJECTIVE: Palatal lift appliances have a role in management of velopharyngeal dysfunction for immobile palates of adequate length where surgery is contraindicated. Conventional appliances involve acrylic/wire work adjustment over successive appointments until they can be tolerated without gagging. A novel appliance has been developed where the lifting plate is incrementally distalized by the patient and vertically adjusted to optimize soft palate positioning. METHOD: The design, construction, and utility of the appliance, which was developed in Dundee Dental Hospital, are described. PARTICIPANTS: The subject was a 12-year-old boy with a variant of Moebius syndrome and velopharyngeal dysfunction. Previous pharyngoplasty had been carried out and further surgery was contraindicated. INTERVENTIONS: The appliance is constructed and fitted and the flexible spring arm is vertically adjusted to lift the soft palate. The screw is turned incrementally at home, extending the lifting plate posteriorly. Videofluoroscopy allows visualization of the appliance and soft palate positioning. MAIN OUTCOME MEASURES/RESULTS: The procedure improved soft palate positioning, as demonstrated by videofluoroscopy, and objective speech outcomes. CONCLUSIONS: The appliance was well tolerated and led to improved speech outcomes for the patient. Adjustments were quick and easy for both clinician and patient. Further studies are needed to definitively determine the efficacy of the appliance.

The challenging management of Moebius syndrome using orthodontic camouflage: A case report.

Moebius syndrome (MS) is a rare congenital neuromuscular disorder characterized by weakness or paralysis (palsy) of abducens and facial nerves, or other cranial nerves which may be affected. Diagnosis, treatment, and dental management of MS patients are focused on treating manifestations like malocclusion, while catering to associated extraoral (neurologic, dermatologic, ocular) complications, aiming to improve their quality of life. Here, we report the case of a 9-year-old female patient with MS who underwent orthodontic camouflage using combined orthopedic-orthodontic therapy using a high-pull chin cup and fixed orthodontic appliance to improve skeletal mal-relation and facial appearance. The outcome displayed great improvement in function and better esthetics, improving not only the patient's but also the family's quality of life. A year's follow-up showed successful maintenance of the achieved results. A multidisciplinary approach in MS not only helps in overcoming the treatment challenges but also provides great psychosocial benefits to these patients.

[Möbius syndrome]. / Le syndrome de Möbius.

Möbius syndrome is a rare congenital disease characterized by facial and abducens nerve palsy. Children are unable to smile, frown, suck, grimace, blink their eyes, and to move their eyes laterally. The aim of this study was to analyze the diagnosis and treatment of this disease. Maxillofacial examination reveals: facial diplegia, retrognathism, palatine and dental malformations. Möbius syndrome is usually associated with multiple cranial nerve involvement, limb or orofacial malformation, and Poland syndrome. Although the pathogenesis of the syndrome is unclear, a number of mechanisms have been suggested including vascular and genetic hypotheses. Symptomatic treatment is used to manage this syndrome. The diagnosis of Möbius syndrome may be difficult in some patients with atypical signs of facial diplegia and other cranial nerve palsies, it requires a multidisciplinary approach.

Clinical characteristics and outcomes of Möbius syndrome in a children's hospital.

BACKGROUND: Möbius syndrome is a congenital disorder with facial and abducens palsy. Although a few case series studies have examined comorbid conditions in Möbius syndrome, follow-up and outcome data are sparse. OBJECTIVES: To examine the clinical characteristics and outcomes of Möbius syndrome. METHODS: Clinical data were reviewed for 10 patients. Neonatal history, neurological examination, comorbid anomalies, medical home care, outcomes, and neuroimaging were summarized. RESULTS: The patients' mean age was 7.3 ± 6.2 years. On neurological examination, absent blink reflex, jaw ankylosis, absent gag reflex, and tongue atrophy were frequently observed. Poland anomaly and clubfoot were present in three and six patients, respectively. Specific therapies required for patients included medical home care (six patients), suction apparatus (six), tube feeding (five), gastrostomy (two), tracheostomy (three), oxygen therapy (three), and home ventilator (two). Punctate calcification in the brainstem was observed in four patients. Pontine and medulla hypoplasia were detected on the basis of anteroposterior diameter in four and seven patients, respectively. Two patients had congenital hydrocephalus with aqueductal stenosis. Global developmental delay occurred in five patients. Three patients died. CONCLUSION: The rate of both the use of home medical devices and death was high in our patients. Möbius syndrome is extremely diverse, not only in clinical manifestation, but also outcome. Early multidisciplinary intervention is important to ensure an optimal outcome. Aqueductal stenosis is an occasional comorbid anomaly resulting from midbrain abnormality.

[Moebius syndrome with facial-dental impairments - rare or rather seldom diagnosed syndrome?]. / Zespól Moebiusa Z Zaburzeniamitwarzowo"zebowymi. Zespól Rzadkowystepujacy, Czy Rzadko Rozpoznawany?

As publications on craniofacial anomalies, malocclusions and dental complications recognised in patients suffering from Moebius syndrome are scarce, the authors of this paper decided to discuss the above aspects in broader terms along with the possibilities offered by orthodontic treatment. The etiology of Moebius syndrome has not hitherto been discovered, however the opinion prevails that it is brought on by multiple factors and conditions. In the analysed case, Moebius syndrome was diagnosed only when the patient was 6 years old. Based on the clinical examination, typical characteristics of the syndrome were observed: craniofacial dysmorphism as well as foot development disorder in the form of talipes equinovarus (club foot). Moreover, Type II Angle's classification of malocclusion was detected - crowded teeth in the mandible and maxilla and hypoplastic enamel. Cephalometric analysis identified retruded position of the mandible against the cranial base, protruded position of the maxilla, shortening of posterior face height, protrusion of incisors in the maxilla. The orthopantomogram showed the presence of all permanent teeth. At the beginning of the orthodontic treatment removable appliances were used, but despite good cooperation on the part of the patient, only a slight improvement was observed. Further orthodontic treatment envisaged extraction of permanent teeth and use of fixed appliances while waiting for the improvement of occlusion.

Tratamientos ortopédicos y ortodóncicos de pacientes con síndromes poco comunes: protocolo / Orthopedic and orthodontic treatments of patients with unusual syndromes: a protocol

Son muchos los síndromes que manifiestan alteraciones dento-esqueletales y, a su vez, manifiestan diferentes complicaciones, no permitiendo tener un protocolo definido para cada síndrome. Para establecer un adecuado protocolo de tratamiento, basado en tratamientos realizados exitosamente y tomando en cuenta los fracasos para no incurrir en el mismo error, se hizo una revisión bibliográfica desdemayo hasta septiembre de 2012, de artículos publicados en los últimos diez (10) años, de revistas internacionales de ortodoncia, ortopedia y cirugía maxilofacial que registraban estudios de investigación y casuística, en buscadores científicos como PubMed, Scielo, Medline. Posteriormente, se tomó la clasificación de Kenneth Lyons Jones, MD, en su obra literaria Patrones Reconocibles de Malformaciones Humanas (2007), tomando en cuenta para la elaboración de este trabajo, los que presentan craneosinostosis, defectos faciales mayores y defectos faciales y de las extremidades como características mayores, que ameritan tratamiento para corregir problemas dento-esqueletales. De los 39 artículos se seleccionaron 11 que tenían relevancia con su tema. Conocer y describir todos los síndromes, mencionando cada característica, es de suma importancia para los profesionales de la salud, ya que de ellos depende no sólo el correcto diagnóstico, sino el tratamiento más adecuado...