Manifestaciones orales del síndrome de Mobius asociado a Poland: descripción de un caso clínico

Avances en odontoestomatologia 01/2009; 25(4). DOI: 10.4321/S0213-12852009000400002
Source: OAI

ABSTRACT The Mobius syndrome is a congenital paralysis of the sixth and seventh cranial nerves. It is characterized by craniofacial and limb malformations. When it is associated to the Poland, the Mobius syndrome shows aplasia of the pectoral muscle (Poland anomaly) and abnormalities of the feet and hands. The syndrome shows important oral and facial abnormalities like gothic palate, open bite, dry mouth, bifid uvula, tongue alterations, and because of this, there is the necessity of an effective odontological approach in order to establish an adequate oral health and patient's quality of life. In this study, the clinical case report shows the oral and general clinical aspects, as well as the odontological treatment made in a patient who has the Mobius/Poland syndrome. After the approach focus on the orientations to the carer and in the control of the periodontal and carie diseases, it was realized a significative reduction in the visible plaque index (VPI) and gum bleeding index (GBI). Currently, the patient is in maintenance. The syndrome is incurable; therefore, the treatment is based on the control and maintenance of oral manifestations. El síndrome de Mobius es una anomalía congénita que causa parálisis de los nervios VI y VII pares craneanos. Se caracteriza por malformaciones límbicas y cráneo-buco-faciales. Asociada a Poland, presenta malformación del músculo pectoral mayor y anomalías en pies y manos. Por ser un síndrome que presenta anomalías faciales y orales importantes como paladar ojival, mordida abierta, xerostomía, úvula bífida, malformaciones en la lengua, se destaca la necesidad de un abordaje odontológico eficaz con el fin de establecer una adecuada salud oral y calidad de vida del paciente. En este trabajo, descripción de un caso clínico, se expone los aspectos clínicos generales y orales, así como el tratamiento odontológico realizado en una paciente portadora del síndrome Mobius/Poland. Un abordaje enfocado en la orientación del responsable y en el control de la caries y la enfermedad periodontal, encontró una reducción significativa en los índices de placa bacteriana y sangrado gingival. Actualmente la paciente se encuentra en fase de mantenimiento. Como no hay cura para el síndrome, el tratamiento es enfocado al control y mantenimiento de las manifestaciones orales.

  • [Show abstract] [Hide abstract]
    ABSTRACT: The aim of this study was to evaluate the orofacial manifestations in patients with Mobius syndrome (MS), establish an early adequate dental treatment and discuss the possible etiology of all cases examined based on information about the gestational intercurrences. Prospective study. Special Care Dentistry Center, School of Dentistry, University of Sao Paulo, Brazil. Subject(s) and methods: Twenty-nine patients with MS aged 0 to 4 underwent prospective dental examination as well as early orthodontic treatment. All patients presented micrognathia, lack of lip seal, high arched palate and weak soft palate. The use of orthopedic appliances was recommended to all 29 patients, but only 13 adhered to treatment and were monitored for at least 24 months. We observed that, after 24 months of treatment, the palate was expanded and micrognathia became less severe in the majority of the cases. Pregnancy-related complications were reported by 27 (97%) of the 29 mothers. The early use of orthopedic appliances was important to prevent malocclusion and glossoptosis. Attempted abortion with misoprostol is associated with an increased risk of MS in infants.
    Oral Diseases 12/2006; 12(6):533-6. DOI:10.1111/j.1601-0825.2006.01231.x · 2.40 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Mobius Syndrome is characterized by showing unilateral facial nerve palsy of the sixth and seventh nerves, lack of facial expression, inability to smile and to tightly close the right eyelids. In this report, a 7-year-old-boy with Mobius syndrome is presented. He had asymmetry of facial expression, anomalies of fingers and severe tooth decay. After dental treatment, the periodic re-care visits should be done according to the eruption pattern.
    The Journal of clinical pediatric dentistry 02/2002; 26(2):207-9. · 0.34 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The combination of Moebius and Poland anomalies is rarely described in the literature. While some authors believe this association is an independent syndrome, others think that Poland, Moebius and Poland-Moebius syndromes are variations of the same condition. We report a case of Poland-Moebius syndrome in a 6-year-old girl who presented with bilateral convergent strabismus, the inability to abduct her eyes beyond the midline and brachydactyly of her right hand. Oral manifestations included incompetent lips and an abnormal tongue. Other facial features included hypoplasia of her mandible and her left ear at a slightly lower level than her right ear. Panoramic and lateral skull radiographs confirmed the absence of certain teeth and the hypoplastic mandible. The diagnosis of Poland-Moebius syndrome was made on the basis of cranial nerve involvement and oro-facial manifestations.
    Oral Diseases 12/2004; 10(6):404-7. DOI:10.1111/j.1601-0825.2004.01045.x · 2.40 Impact Factor


Available from