IADR Abstract Archives
Review: Orofacial Considerations in Dental Management of Fragile X Children
Objectives: Fragile-X syndrome (FXS) is characterized by an intellectual disability associated with an X-linked dominant inheritance pattern. It is described as the second most prevalent syndrome in the world after Down syndrome. Even though it is relatively frequent, literature on dental and orthodontic management is scarce. This review is proposed to characterize patients with SXF from an orofacial and behavioral point of view and how it can influence the therapy.
Aim:To describe orofacial dysfunctions and musculoskeletal characteristics of SXF and therapeutic management at early ages.
Methods: Bibliographic review in PUBMED and SCIELO database. Information was sought from March-June 2017, including only manuscripts from the last 5 years. The terms used were: X-fragil syndrome, orthodontic treatment, orthopedic treatment, dental treatment and children.
Results: Orofacial characteristics exhibited by these patients are: elongated faces, hypoplasia of the middle third of the face, forehead and prominent brows, sunken eyes and large ears with low insertion. Dento-skeletally, these patients have been described as having deep palate, prominent mandible, increased mandibular angle, single or bilateral crossbites, enamel hypoplasias, dental anomalies of shape and number. Orofacial functions alterations like atypical swallowing, dysphagia, oral breathing, and obstructive sleep apnea are associated with reduced muscle tone. Regarding behavioral management, it is described that these patients present sensory integrative disorders, cognitive and attention deficits. These problems originate from orofacial alterations that impede the correct development of speech / language and learning. There is no documented cases of orthodontic or orthopedic treatment for the management of dento-skeletal disorders, however, a case of myofunctional therapy treatment for muscular re-education is presented.
Conclusions: SXF has multiple presentations and it is important to adapt dental treatment to each case. Considering the characteristics presented in this review focusing on the prevention of orofacial pathologies, reeducation of altered functions, prevention and interception of dentomaxillary anomalies, using appropriate strategies for the cognitive conditions and comorbidities in each case, considering an interdisciplinary approach.
Aim:To describe orofacial dysfunctions and musculoskeletal characteristics of SXF and therapeutic management at early ages.
Methods: Bibliographic review in PUBMED and SCIELO database. Information was sought from March-June 2017, including only manuscripts from the last 5 years. The terms used were: X-fragil syndrome, orthodontic treatment, orthopedic treatment, dental treatment and children.
Results: Orofacial characteristics exhibited by these patients are: elongated faces, hypoplasia of the middle third of the face, forehead and prominent brows, sunken eyes and large ears with low insertion. Dento-skeletally, these patients have been described as having deep palate, prominent mandible, increased mandibular angle, single or bilateral crossbites, enamel hypoplasias, dental anomalies of shape and number. Orofacial functions alterations like atypical swallowing, dysphagia, oral breathing, and obstructive sleep apnea are associated with reduced muscle tone. Regarding behavioral management, it is described that these patients present sensory integrative disorders, cognitive and attention deficits. These problems originate from orofacial alterations that impede the correct development of speech / language and learning. There is no documented cases of orthodontic or orthopedic treatment for the management of dento-skeletal disorders, however, a case of myofunctional therapy treatment for muscular re-education is presented.
Conclusions: SXF has multiple presentations and it is important to adapt dental treatment to each case. Considering the characteristics presented in this review focusing on the prevention of orofacial pathologies, reeducation of altered functions, prevention and interception of dentomaxillary anomalies, using appropriate strategies for the cognitive conditions and comorbidities in each case, considering an interdisciplinary approach.