Jaw malformations plus displacement and numerical aberrations of teeth in neurofibromatosis type 1
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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. / Friedrich, Reinhard E; Giese, Manfred; Schmelzle, Rainer; Mautner, Victor-Felix; Scheuer, Hanna A.
in: J CRANIO MAXILL SURG, Jahrgang 31, Nr. 1, 01.02.2003, S. 1-9.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Jaw malformations plus displacement and numerical aberrations of teeth in neurofibromatosis type 1
T2 - a descriptive analysis of 48 patients based on panoramic radiographs and oral findings
AU - Friedrich, Reinhard E
AU - Giese, Manfred
AU - Schmelzle, Rainer
AU - Mautner, Victor-Felix
AU - Scheuer, Hanna A
PY - 2003/2/1
Y1 - 2003/2/1
N2 - 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.
AB - 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.
KW - Adolescent
KW - Adult
KW - Aged
KW - Anodontia
KW - Child
KW - Child, Preschool
KW - Cranial Nerve Neoplasms
KW - Dental Models
KW - Female
KW - Humans
KW - Jaw Abnormalities
KW - Jaw Neoplasms
KW - Male
KW - Malocclusion
KW - Mandibular Nerve
KW - Middle Aged
KW - Molar
KW - Neoplasm Invasiveness
KW - Neurofibroma, Plexiform
KW - Neurofibromatosis 1
KW - Photography
KW - Physical Examination
KW - Radiography, Panoramic
KW - Tooth Abnormalities
KW - Trigeminal Nerve Diseases
M3 - SCORING: Journal article
C2 - 12553919
VL - 31
SP - 1
EP - 9
JO - J CRANIO MAXILL SURG
JF - J CRANIO MAXILL SURG
SN - 1010-5182
IS - 1
ER -