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Jaw malformations plus displacement and numerical aberrations of teeth in neurofibromatosis type 1: a descriptive analysis of 48 patients based on panoramic radiographs and oral findings
- Source :
- Journal of Cranio-Maxillofacial Surgery. 31:1-9
- Publication Year :
- 2003
- Publisher :
- Elsevier BV, 2003.
-
Abstract
- Aim: The aim of this study was to analyse jaw malformations and tooth displacement in patients with neurofibromatosis type 1 (NF1). Material and Methods: Forty-eight patients were included in the study (male or female 24 each). All fulfilled the current NIH diagnostic criteria for NF1. The age range was 2.5–66 years. The type of neurofibroma was histologically proven in surgically treated patients. Patients with disseminated cutaneous neurofibromata and those with the plexiform type were distinguished. The analysis was based on physical investigation, photographs, panoramic radiographs and dental casts. Results: With the emphasis on alterations of tooth position, deformities of the adjacent bones and malocclusion, the majority of these patients (26) were affected by plexiform neurofibromata. In the other 22 patients with disseminated neurofibromata, malformations of the alveolar ridge were absent and individual oral symptoms were rarely found and were mild, and in all cases were unimpaired. Numerical aberrations and retention of molars was exclusively associated with a trigeminal nerve affected by plexiform neurofibroma. Aplasia of a second lower molar was recognized in four of these plexiform-neurofibroma patients. Conclusion: It is widely accepted that malformations of the facial skeleton are often of genetic origin. However, in this study these malformations were strongly associated with plexiform neurofibromata originating from the trigeminal nerve. Thus, in addition to presently unknown genetic factors, the pattern of skeletal malformation can be caused by tumour invasion and local destruction, e.g. the neuromuscular unit or prenatal development of the plexiform neurofibroma in the inferior alveolar nerve. It is further concluded that epidemiologic studies on the incidence and severity of NF1 in the oral and maxillofacial region have to distinguish between patients with or without plexiform neurofibroma, especially when analysing alterations and deformities of the jaws, teeth and malocclusion. Aplasia of second inferior molars is an additional (dental) finding associated with plexiform neurofibromata in NF1.
- Subjects :
- Adult
Male
Neurofibromatosis 1
Adolescent
Mandibular Nerve
Mandibular nerve
Inferior alveolar nerve
Jaw Abnormalities
stomatognathic system
Plexiform neurofibroma
Radiography, Panoramic
Photography
medicine
Alveolar ridge
Humans
Neurofibroma
Cranial Nerve Neoplasms
Neoplasm Invasiveness
Jaw abnormality
Neurofibromatosis
Child
Physical Examination
Aged
Anodontia
Neurofibroma, Plexiform
Tooth Abnormalities
business.industry
Anatomy
Middle Aged
medicine.disease
Jaw Neoplasms
Molar
Models, Dental
stomatognathic diseases
Otorhinolaryngology
Trigeminal Nerve Diseases
Child, Preschool
Female
Surgery
sense organs
Oral Surgery
business
Malocclusion
Subjects
Details
- ISSN :
- 10105182
- Volume :
- 31
- Database :
- OpenAIRE
- Journal :
- Journal of Cranio-Maxillofacial Surgery
- Accession number :
- edsair.doi.dedup.....93844ee9fd074c0b6e0b775b4199c369