Oral clinical manifestations of neurofibromatosis type 1 in children and adolescents
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1
Second University of Naples, Multidisciplinary Department of Medical-Surgical and Dental Specialties, Italy
Aim.
The purpose of the present study is to investigate the prevalence of oral manifestations in a group of patients affected by neurofibromatosis type 1, comparing it with that of a group of healthy patients similar for age and sex.
Materials and Methods.
100 patients, between 2 and 18 years, with genetically confirmed NF1 from the Pediatric Department of the University of Campania “Luigi Vanvitelli” were enrolled in this study. A total of 100 pediatric patients with no history of neurofibromatosis type 1 constituted the control group and were matched to cases for age and sex. Clinical examinations were conducted for both groups and a reproducible procedure was used to investigate: dental caries, dental abnormalities, periodontal health, neurofibromas, malocclusions and enamel defects. In the evaluation of DMFT/dmft Student’s t-test was used, whereas Chi-squared test was used to evaluate other kinds of manifestations.
Results.
Student’s t-test concerning prevalence of dental caries resulted in a significant difference between the two groups (NF1 patients DMFT = 3.0, control group DMFT = 5.24; p < 0.01); a significant difference was highlighted as regards the other kinds of manifestations as well: dental abnormalities (NF1 patients: 21%, control group: 8%; p = 0.009), enamel defects (NF1 patients: 36%, control group: 14%; p = 0.003), neurofibromas (NF1 patients: 4%, control group: 0%; p = 0.0043) and malocclusions (NF1 patients: 52%, control group: 34%; p = 0.01).
Discussion.
Most of scientific literature deals with NF1-related oral manifestations in adults and it is poor of data concerning oral manifestations of NF1 in children. Data obtained in this study with regard to dental caries do not confirm a correlation between tooth decay and NF1, in particular DMFT/dmft index of NF1 patients is lower than the control group one. Remarkable data were obtained as regards enamel defects because this condition was reported to occur more frequently in NF1 patients. A similar increased frequency was also observed regarding NF1 with dental abnormalities, some of which were related to the presence of neurofibromas. There are few available data about the presence of neurofibromas in children. However, in the present study, perioral and multiple head and neck neurofibromas with implications in the oral cavity were observed: as a result, a statistically significant difference was found between NF1 patients and the control group. Scientific literature is poor of data concerning this kind of manifestation in children with NF1 even for malocclusions. This study found a statistically significant difference as malocclusions proved to be a frequent condition in NF1-affected children. According to data obtained from the present study, it can be stated that NF1-ralated oral manifestations can be detected during childhood and adolescence. Although the presence of neurofibromas is the most distinctive and best-known condition associated with NF1, these lesions were underrepresented in the sample examined. Pediatric dentists ought to be aware of the possible involvement of the oral cavity in order to prevent complications and to properly set up a treatment plan, particularly with regard to the presence of enamel defects and malocclusions.
References
1. Bardellini E, Amadori F, Flocchini P, Conti G, Piana G, Majorana A. Oral findings in 50 children with neurofibromatosis type 1. A case control study. Eur J Paediatr Dent 2011;12:256–60.
2. D’Ambrosio JA, Langlais RP, Young RS. Jaw and skull changes in neurofibromatosis. Oral Surg Oral Med Oral Pathol 1988;66: 391–6.
3. Javed F, Ramalingam S, Ahmed HB, Gupta B, Sundar C, Qadri T, Al-Hezaimi K, Romanos GE. Oral manifestations in patients with neurofibromatosis type-1: a comprehensive literature review. Crit Rev Oncol Hematol. 2014 Aug;91(2):123-9.
Keywords:
Dental Caries,
Dental abnormalities,
Neurofibromas,
Malocclusions,
Enamel defects
Conference:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine., Ancona, Italy, 19 Oct - 20 Oct, 2018.
Presentation Type:
Poster Presentation
Topic:
Oral Diseases
Citation:
Di Petrillo
M,
Loffredo
F,
Romano
A,
Santoro
R,
Santoro
C and
Lucchese
A
(2019). Oral clinical manifestations of neurofibromatosis type 1 in children and adolescents.
Front. Physiol.
Conference Abstract:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine..
doi: 10.3389/conf.fphys.2019.27.00015
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Received:
11 Oct 2018;
Published Online:
09 Dec 2019.
*
Correspondence:
Dr. Marco Di Petrillo, Second University of Naples, Multidisciplinary Department of Medical-Surgical and Dental Specialties, Naples, Campania, 80138, Italy, marcomascitti86@hotmail.it