Fragile X syndrome: panoramic radiographic evaluation of dental anomalies, dental mineralization stage, and mandibular angle

Abstract

Fragile X syndrome (FXS) is a disorder linked to the chromosome X long arm (Xq27.3), which is identified by a constriction named fragile site. It determines various changes, such as behavioral or emotional problems, learning difficulties, and intellectual disabilities. Craniofacial abnormalities such as elongated and narrow face, prominent forehead, broad nose, large and prominent ear pavilions, strabismus, and myopia are frequent characteristics. Regarding the oral aspects, deep and high-arched palate, mandibular prognathism, and malocclusion are also observed.Objective:The purpose of this study was to evaluate the dental radiographic characteristics as described in 40 records of patients with panoramic radiography.Material and Methods:The patients were in the range of 6–17 years old, and were divided into two groups (20 subjects who were compatible with the normality standard and 20 individuals diagnosed with the FXS), which were matched for gender and age. Analysis of the panoramic radiographic examination involved the evaluation of dental mineralization stage, mandibular angle size, and presence of dental anomalies in both deciduous and permanent dentitions.Results:The results of radiographic evaluation demonstrated that the chronology of tooth eruption of all third and second lower molars is anticipated in individuals with FXS (p<0.05). In this group, supernumerary deciduous teeth (2.83%), giroversion of permanent teeth (2.31%), and partial anodontia (1.82%) were the most frequent dental anomalies. In addition, an increase was observed in the mandibular angle size in the FXS group (p<0.05).Conclusion:We conclude that knowledge of dental radiographic changes is of great importance for dental surgeons to plan the treatment of these individuals.

Symptomatic Mandibular Fibrous Dysplasia With Concurrent Triple X- and Premutation Stage Fragile-X-Syndrome: Case Report With Short Literature Survey

“…The dental findings are abnormalities of root morphology, the comparatively early emergence of molars, and a larger jaw angle. However, the correlations of the findings were generally low (122). Earlier studies had shown significantly more frequent malocclusions but no differences in jaw relations and palatal dimensions in FXS (123).…”

“…Dentition. In one study, numerical and structural changes in teeth were demonstrated in FXS (122). The dental findings are abnormalities of root morphology, the comparatively early emergence of molars, and a larger jaw angle.…”

Impacto en la calidad de vida de los tratamientos odontológicos bajo anestesia general en pacientes con discapacidad intelectual

“…Aunque la prevalencia es mayor en hombres que en mujeres, los casos que se producen en las mujeres tienen menores afectaciones clínicas. Es el responsable de alteraciones sistémicas, problemas de comportamiento, dificultad de aprendizaje y retraso mental [141,142].…”

“…Físicamente, los individuos afectados por este síndrome tienen unas características tales como cara larga, orejas prominentes, estrabismo, "pectus excavatum", aumento del tamaño testicular y prolapso de la válvula mitral. Además, a nivel psicológico presentan retraso mental, características autísticas e hiperactividad [141,142].…”

“…A nivel oral podemos encontrarnos con un paladar estrecho y ojival, prognatismo, macroglosia, déficit de higiene oral, hipoplasia del esmalte, maloclusiones (mordida cruzada y abierta), hipodoncia, alteraciones de forma dentaria, macrodoncia, caninos incluidos, caries y gingivitis [141,142]. En la Figura 7 podemos observar una imagen intraoral de un paciente con S. X Frágil tras una profilaxis, donde observamos la malposición dentaria.…”