Occlusal traits in children with neurofibromatosis type 1

Bardellini, Elena; Tonni, Ingrid; Micheli, Roberto; Molinaro, Anna; Amadori, Francesca; Flocchini, Pierangela; Piana, Gabriela; Majorana, Alessandra

doi:10.1111/ocr.12108

Cited by 16 publications

(31 citation statements)

References 28 publications

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“…NF-1 oral mucosal pathology is defined by the presence of soft tissue tumor (Figure 2 and Figure 3). In the oral cavity, nodular neurofibromas of the tongue are most common [34]. There, they lead to macroglossia and enlargement of the fungiform papillae [26] [35] [36].…”

Section: Oral Mucosal Pathologymentioning

confidence: 99%

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Oral Manifestations of Neurofibromatosis Type 1

Chérifi¹,

Fournier²,

Berdal³

et al. 2019

JCDSA

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Neurofibromatosis type 1 (NF-1) is a common genetic disorder with a highly variable phenotype. The disease affects both proliferation and differentiation of cells of neurectodermal origin. The presence of tumors is very common like benign nodular neurofibromas. Tumors with unclear prognosis may be present like plexiform neurofibromas whose prognosis is more uncertain. While many organs exhibit pathologies, most commonly affected are the nervous system, skin, gastrointestinal tract and heart. Oral abnormalities are also very common: 72% of NF-1 patients exhibit pathologies in oral mucosa, gums, maxillary and temporomandibular joints, and teeth. The incidence of NF-1 and its relationship to the prevalence of caries have been discussed in other researches. It is known that poor oral hygiene plays a key role in the development of periodontal disease and caries. Here we review the oral manifestations of neurofibromatosis type 1 that we illustrate by a patient followed in the center of rare diseases of the hospital Henri Mondor, clinical service in which we work.

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Section: Oral Mucosal Pathologymentioning

confidence: 99%

“…The absence of second molars of patients with plexiform neurofibromas causes asymmetries that can affect the temporomandibular joint [62]. For the orthodontic abnormalities, only Class III molar occurrence is higher [34]. For children, development of primary teeth can be earlier in NF-1 patients [63].…”

Section: Dental Abnormalities and Cariesmentioning

confidence: 99%

Oral Manifestations of Neurofibromatosis Type 1

Chérifi¹,

Fournier²,

Berdal³

et al. 2019

JCDSA

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“…We find significant and robust changes in the nasal region of Nf1ob-/-mice. This pattern of deformed frontal midface region of the skull in Nf1ob-/-mice parallels the quantitative changes in the analogous structures in human NF1 craniofacial skeleton resulting in unilateral proptosis of an eye and facial asymmetry [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26]. NF1 patients often exhibit macrocephaly.…”

Section: Bmentioning

confidence: 99%

“…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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“…The world's scientific literature is replete with present relevant researches on caries in children 4,5 . However, there is a big gap when we observe the low scientific productivity of oral lesions on soft tissue, affecting the oral health of pediatric patients 4,[6][7][8] . It is essential to the affirmation of the high incidence of these lesions in children; and the certainty that, in most cases, these injuries do not get a quick diagnosis that enables a ready treatment 9,10 .…”

Section: Introductionmentioning

confidence: 99%

Lesões bucais na infância: revisão sistemática de interesse da fonoaudiologia

Matos

Sá²,

Pereira³

et al. 2016

Rev. CEFAC

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The background of this study is speech therapists, physicians and dentists that are professionals that in their professional actions, conduct intraoral examinations on their patients. Many children, from birth, have affection for oral lesions that can bring temporary inconvenience to daily life. The purpose is to provide health professionals a systematic review about the most common oral lesions in children and the interests of speech therapy. Health professionals should always seek to increase their knowledge by improving health care for their patients. Keywords: Oral Health; Stomatitis; Mucocele RESUMO Este estudo tem como tema, fonoaudiólogos, médicos e dentistas que são profissionais que, frequentemente, em suas ações profissionais, realizam exames intrabucais em seus pacientes. Muitas crianças, desde seu nascimento, apresentam o acometimento por lesões bucais que podem trazer transtornos temporários ao cotidiano. O objetivo é apresentar aos profissionais de saúde uma revisão sistemática acerca das lesões bucais mais frequentes em crianças e de interesse da fonoaudiologia. Os profissionais de saúde devem buscar sempre aumentar seus conhecimentos melhorando a atenção à saúde de seus pacientes.

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Occlusal traits in children with neurofibromatosis type 1

Abstract: An early orthodontic evaluation might be planned in the management of children with NF1 to prevent or decrease the need for extensive orthodontic interventions.

Cited by 16 publications

References 28 publications

Oral Manifestations of Neurofibromatosis Type 1

Oral Manifestations of Neurofibromatosis Type 1

An Osteoblast Origin for Craniofacial Dysplasia in Neurofibromatosis Type I

Lesões bucais na infância: revisão sistemática de interesse da fonoaudiologia

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