Jaw malformations plus displacement and numerical aberrations of teeth in neurofibromatosis type 1

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 -