Oral Clinical Manifestations of Neurofibromatosis Type 1 in Children and Adolescents
2020
Rossella Santoro | Claudia Santoro | Francesca Loffredo | Antonio Romano | Silverio Perrotta | Rosario Serpico | Dorina Lauritano | Alberta Lucchese
<b>Background:</b> Neurofibromatosis 1 (NF1) is an autosomal dominant genetic disorder. The expression of NF1 is extremely variable considering the broad spectrum of mutations affecting the gene(s) responsible for the pathology. <b>Aim:</b> To investigate the prevalence of oral manifestations in a group of children affected by neurofibromatosis type 1. <b>Design:</b> 100 pediatric patients, with genetically confirmed NF1 were enrolled in this study and matched to a total of 100 healthy children. Clinical examination was used to investigate: dental caries, dental abnormalities, periodontal health, neurofibromas, malocclusions, and enamel defects. <b>Results:</b> Mann Whitney’s test concerning prevalence of dental caries resulted in a no significant difference between the two groups (<i>p</i> = 0.90); a significant difference was highlighted as regards the other kinds of manifestations as well: enamel defects (<i>p</i> = 0.01), neurofibromas (<i>p</i> = 0.0043) and poor oral hygiene (<i>p</i> = 0.0002) with a higher prevalence of these features in NF1 patients than healthy controls. Similar results come out, regarding dental abnormalities in which can observe a significant difference between shape anomalies (<i>p</i> < 0.001). <b>Conclusion:</b> According to data obtained from the present study, it can be stated that NF1-related oral manifestations can be detected during childhood and adolescence. In particular for neurofibromas, enamel defects, shape anomalies, and poor oral hygiene.
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