Oral findings in 50 children with neurofibromatosis type 1. A case control study
E Bardellini1, F Amadori, P Flocchini
1University of Brescia, Dental Clinic, Brescia, Italy. bardalena@libero.it
Insights
Children with Neurofibromatosis type 1 (NF1) exhibit distinct oral health issues, including poor hygiene and jaw abnormalities. Early monitoring of oral manifestations in pediatric NF1 patients is crucial for long-term health outcomes.
Area of Science:
- Pediatric Dentistry
- Genetics
- Oral Medicine
Background:
- Limited data exists on oral manifestations in children with Neurofibromatosis type 1 (NF1).
- NF1 is a genetic disorder affecting nerve tissue growth, with potential oral implications.
- Understanding early oral signs in pediatric NF1 is essential for timely intervention.
Purpose of the Study:
- To investigate and document the oral findings in a cohort of children diagnosed with NF1.
- To compare oral health parameters in children with NF1 against a control group.
- To identify specific dental and craniofacial characteristics associated with NF1 in childhood.
Main Methods:
- A clinical research study involving 50 children with NF1 and a control group.
- Comprehensive examinations of craniofacial structures, dental status, and oral tissues.
- Assessment of dental caries, malocclusions, periodontal health, neurofibromas, and jaw abnormalities.
- Statistical analysis using Unpaired Student's t test and Fisher's test (p <0.05 significance).
Main Results:
- Children with NF1 demonstrated significantly poorer oral hygiene compared to controls.
- Increased prevalence of Class III dental malocclusions was observed in the NF1 group.
- Severe alterations in the mandible and temporomandibular joint were noted in children with NF1.
Conclusions:
- Oral manifestations of NF1 are present in childhood, even before the development of classic neurofibromas.
- Long-term follow-up is recommended for pediatric NF1 patients to monitor potential oral lesion development.
- Early identification of oral findings in NF1 can guide management and improve patient outcomes.
Aim:
The scientific literature is poor of data concerning NF1-related oral manifestations in children. The purpose of this study was to investigate the oral findings in a group of children with NF1.
Materials And Methods:
This study was designed as a clinical research. A total of 50 children affected by NF1 were enrolled. Non-NF1 controls were selected among subjects referred to the Paediatric Dentistry Department of the Dental Clinic at the University of Brescia, Italy. The craniofacial and all the dental examinations were carried out. The following parameters were investigated: dental caries, dental abnormalities, periodontal status, oral and perioral neurofibromas, orthodontic features, mandible and temporomandibular joint abnormalities. Statistics Unpaired Student's t test was calculated for Gaussian distributed variables; Fisher's test for non-Gaussian distributed binomial variables was used. The values inferior to p <0.05 (threshold) were considered significant for the study.
Results:
Children with NF1 showed poorer oral hygiene conditions, more frequent Class III dental malocclusions and severe alterations of the mandible and the temporal mandibular joint, if compared with the control group.
Conclusion:
NF1 related-oral manifestations are evidenced also during childhood, even if some of the best known, like the neurofibromas, are not yet developed. It is important to keep a long-term follow-up to monitor the possible development of other oral lesions.
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