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, R.; Mautner, Victor‐Felix; Scheuer, Hanna A

doi:10.1016/s1010-5182(02)00160-9

Cited by 83 publications

(114 citation statements)

References 31 publications

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“…This could be understandable in cases associated with other facial abnormalities, but in a case with a high number of isolated supernumerary teeth, the mechanism is far from being understood. Hyperdontia when described in NF1 [6,10,[16][17][18] only appears as retained or impacted teeth, as in the case of our patient.…”

Section: Discussionsupporting

confidence: 68%

“…Most articles considering NF1 describe missing teeth [4,6]. Hyperdontia is poorly described, especially as an isolated facial manifestation, as in this case report.…”

Section: Discussionmentioning

confidence: 74%

“…Freeman and Standish [9] and Shapiro et al [5] first mentioned hyperdontia with NF1. The presence of supernumerary teeth is more likely associated with other facial abnormalities, such as a tumor-like plexiform neurofibroma [6,[16][17][18]. Most articles hypothesize that the missing or supernumerary tooth are related to local tumor expression of NF1.…”

Section: Discussionmentioning

confidence: 99%

“…Many multisystemic complications such as cerebral tumors or vasculopathies could lead to death [1,2]. Oral and maxillofacial manifestations may also be found in patients with NF1, such as gingival enlargement and pigmentation, oral and perioral neurofibroma, osseous lesions of the maxilla or the mandible, and dental abnormalities [3][4][5][6][7][8][9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

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Neurofibromatosis type I and supernumerary teeth: up-to date review and case report

Cervellera

Dissaux³

2016

Med Buccale Chir Buccale

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-Introduction: Neurofibromatosis type I (NF1) is a genetic disease that may involve oral manifestations such as supernumerary teeth. Case report: A 17-year-old male patient with NF1 was referred by an orthodontist for avulsion of wisdom teeth and six supernumerary teeth. Ten teeth were removed under general anesthesia in December 2015. Discussion: Management of supernumerary teeth is very controversial and patient care of NF1 is complex. Conclusion: Even though the presence of supernumerary teeth in cases of NF1 is not well-known, patient care is extremely important and requires a team of specialists.Résumé -Les dents surnuméraires dans la neurofibromatose de type I : cas clinique et revue de la littéra-ture. Introduction : La neurofibromatose de type I est une maladie génétique qui peut induire des manifestations orales comme la présence de dents surnuméraires. Observation clinique : Un homme de 17 ans présentant une NF1 a été adressé par son orthodontiste pour l'avulsion de ses dents de sagesse et 6 dents surnuméraires. Les 10 dents ont été extraites sous anesthésie générale en décembre 2015. Discussion : La prise en charge des dents surnuméraires est très controversée et la prise en charge des patients atteints de NF1 est complexe. Conclusion : Même si la présence de dents surnuméraires chez les patients atteints de NF1 est mal connue, la prise en charge de ces patients est très importante et une équipe pluridisciplinaire est nécessaire.

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Section: Discussionsupporting

confidence: 68%

“…Most articles considering NF1 describe missing teeth [4,6]. Hyperdontia is poorly described, especially as an isolated facial manifestation, as in this case report.…”

Section: Discussionmentioning

confidence: 74%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Neurofibromatosis type I and supernumerary teeth: up-to date review and case report

Cervellera

Dissaux³

2016

Med Buccale Chir Buccale

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“…Other craniofacial findings have been reported in NF1 including a widening of inferior alveolar canal, an enlarged mandibular foramen, and a reduction in mandibular angle. In rare cases plexiform Neurofibromas have been reported to be associated with impacted teeth and deformed alveolar ridge while optic nerve gliomas have been associated with orbit defects [12][13][14][15]. NF1 patients may also display generalized defects in bone metabolism and bone turnover, resulting in osteoporosis in up to one half of affected individuals [16,17].…”

Section: Introductionmentioning

confidence: 99%

An Osteoblast Origin for Craniofacial Dysplasia in Neurofibromatosis Type I

Bamaga¹,

O'Sullivan²,

Schmitz³

et al. 2017

JDHODT

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Sphenoid wing dysplasia (SWD), which results in a craniofacial deformity, is the third most common skeletal deformity in Neurofibromatosis Type I (NF1) patients. A neurofibromin osteoblast conditional deletion mouse model (Nf1ob-/-) has been developed to study the skeletal abnormalities in NF1. No overt morphological phenotype is seen in the appendicular or axial skeleton of these mice. Nf1ob-/-mice have been shown to have increased bone porosity and lower bone density. We noted a progressive craniofacial deformity, which results in cranial asymmetry, malocclusion and unilateral proptosis of an eye. To assess this deformity, we employed micro-computed tomography (µCT) and geometric morphometric analysis to compare Nf1ob-/-mouse skulls to control animals. Landmarks were placed on the images at 13 biologically relevant cranial anatomical sites. Analysis of distances and angles between these landmarks revealed that there is significant variation between the Nf1ob-/-mice and controls. We found that the nasal and frontal bones of Nf1ob-/-mice skulls are deviated from the central line, whereas it was straight in controls. The nasal region is tipped downward in Nf1ob-/-mice. We also noted that the cranium shows a trend toward macrocephaly in Nf1ob-/-mice whereas other measurements were within the range of normal control mice. These differences correspond to those seen in craniofacial dysplasia and SWD in NF1 patients. No tumors were found associated with the craniofacial dysplasia in Nf1ob-/-mice. Our results identify a primarily osteoblast origin for the pathogenesis of craniofacial dysplasia in Nf1ob-/-mice and strongly support an osteoblast origin for SWD in NF1. In addition, we identify and validate a novel mouse model with which to explore molecular mechanisms and test preventative treatments for SWD in NF1.

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