Dental characteristics of fibrous dysplasia and McCune-Albright syndrome

Akintoye, Sunday O.; Lee, Janice S.; Feimster, Tawana; Booher, Susan; Brahim, Jaime S.; Kingman, Albert; Riminucci, Mara; Robey, Pamela Gehron; Collins, Michael T.

doi:10.1016/s1079-2104(03)00225-7

Cited by 82 publications

(74 citation statements)

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“…According to Akintoye et al 9 , it is a challenge to perform orthodontic treatments in patients with FD and therefore special care should be paid to the close relationship between lesion and teeth. FD is a rare but potentially severe disease which can cause fracture when affecting long bones, including osseous pain, deformities and compression of surrounding tissues 1,7 .…”

Section: Discussionmentioning

confidence: 99%

“…Clinically, FD can be classified as monostotic when limited to only one bone and polyostotic when more bones are involved [1][2][5][6][7][8][9][10][11][12][13] . The monostotic FD is the most common form, representing 70 to 85% of the cases 7,13 and mainly occurring in the bones of the face, with the maxilla being more frequently affected than the mandible [1][2]8,10,[14][15][16] .…”

Section: Introductionmentioning

confidence: 99%

“…asymmetry which may cause severe deformity resulting in functional and aesthetic compromise 6,20 . Craniofacial FD may also be associated with dental problems, such as malocclusion 4,9,16,[20][21] , changes in tooth positioning and prolonged retention of deciduous teeth (9), thus affecting mastication and speech 21 . Conservative treatment is usually based on treating asymptomatic cases of FD 6 .…”

Section: Introductionmentioning

confidence: 99%

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Monostotic fibrous dysplasia: a case report with cone-beam computed tomography findings

Costa

Peyneau

Verner

et al. 2017

RGO, Rev. Gaúch. Odontol.

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Fibrous dysplasia is a benign fibrous-osseous lesion in which normal RESUMOA displasia fibrosa é uma lesão fibro-óssea benigna em que o osso normal é substituído por tecido conjuntivo fibroso e osso imaturo, podendo afetar um único osso (monostótica) ou vários ossos (poliostótica), ocorrendo principalmente em crianças e adultos jovens. Quando presente nos ossos faciais, a maxila é envolvida com mais frequência que a mandíbula, podendo causar assimetria facial, além de complicações odontológicas. Nos exames por imagem a característica principal da displasia fibrosa é a aparência de vidro despolido. A tomografia computadorizada é o método ideal para avaliação dessa lesão e relação com estruturas adjacentes. A radiografia convencional, devido a sobreposição de estruturas anatômicas, torna difícil a delimitação da extensão da lesão. O presente estudo teve como objetivo apresentar o relato de caso de um paciente do sexo feminino, 10 anos de idade, com presença de displasia fibrosa na maxila, objetivando orientar os cirurgiões-dentistas com relação às principais características imaginológicas dessa lesão.Termos de indexação: Tomografia computadorizada de feixe cônico. Displasia fibrosa monostótica. Maxila.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Monostotic fibrous dysplasia: a case report with cone-beam computed tomography findings

Costa

Peyneau

Verner

et al. 2017

RGO, Rev. Gaúch. Odontol.

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“…Regular clinical check-ups are recommended as the disease is associated with enamel hypoplasia, dentin dysplasia, taurodontism, an increased caries index score, and malocclusion [21]. Orthodontic therapy is described in the literature as more prolonged, but this is not a contraindication [22].…”

Section: Discussionmentioning

confidence: 99%

Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Angermair¹,

Fretwurst²,

Semper-Hogg³

et al. 2017

Dentistry

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and sclerotic areas. These variable radiographic presentations impede radiographic diagnostics and require for an experienced practitioner to plan correct patient management Therefore, the present case report demonstrates the strongly deviating clinical and radiological manifestations of monostotic and polyostotic forms of fibrous dysplasia (FD) in the facial area and its impact on dental and surgical therapy. Presentation of Case Patient 1A 14-year-old male patient was admitted to the Department of Oral-and Craniomaxillofacial Surgery presenting a painless distension in the anterior lower jaw which appeared approximately 4 month before dental visit. Clinical diagnostics revealed an inconspicuous, pale reddish gingiva in the concerned region. In the right incisor region, a hard, non-compressive, non-displaceable swelling of 4 × 3 cm (Figure 1) was detectable. The patient demonstrated a conservative adequately restored juvenile dentition and good oral hygiene behavior with slightly soft plaque caused by orthognathic multiband brackets. The teeth showed neither loosening nor percussive sensitivity. In Keywords: Fibrous dysplasia; Craniofacial fibrous dysplasia;McCune-Albright syndrome; Cone beam computer tomography; Radiographic diagnostics IntroductionFibrous dysplasia (FD) is a rare, congenital malformation of the bone tissue. The disease occurs as a monostotic form, in which only a single bone is affected, and a polyostotic form involving multiple skeletal locations. Fibrous dysplasia may also occur within the context of McCune-Albright Syndrome, a severe polyostotic disease in which endocrine disorders (e.g., puberty praecox, hyperthyreodism, Cushing's disease) and pigmentation of the skin ("cafe au lait" spots) are present [1,2]. While fibrous dysplasia accounts for approximately 7% of all benign osseous tumors, McCune-Albright syndrome occurs very rarely, with a prevalence of 1/100,000 to 1/1,000,000 [3]. In 70-80% of the cases the disease manifests in a monostatic form in the femur, tibia, and ribs, and in 20-30% as a polyostotic form [4].The pathology is a mutation of the GNAS gene on chromosome 20 in early embryogenesis [5]. This gene encodes a G protein, which leads to dysregulation of the adenylyl cyclase [6]. Overproduction of cyclic adenosine monophosphate (cAMP) leads to a deficient differentiation of bone stem cells [7] and finally to the replacement of the healthy bone matrix by proliferating bone marrow stromal cells. This leads to the formation of the characteristic fibrous lesions [8,9]. Histological lesions are comprised of irregular trabecular formation within a fibrous stroma with bone marrow stromal cells without differentiation in adipocytes and osteoblasts. The bony matrix is replaced by proliferating soft tissue and shows an irregular woven structure that is described to resemble "Chinese character" pattern [8].These changes in the bony structure lead to a characteristic radiological manifestation of the disease in juvenile patients where a "ground glass"-like radiolucency enables a tenta...

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“…(10) Taurodontism occurs mostly as an isolated anomaly, but some developmental syndromes and anomalies like amelogenesis imperfecta (AI), Down's syndrome, Ectodermal dysplasia, Klinefelter syndrome, Trichodento-osseous syndrome, Mohr syndrome, WolfHirschhorn syndrome and Lowe syndrome have shown their association with it. (11) In some rare syndromes like Williams syndrome, (12) McCune-Albright syndrome, (13) Smith-Magenis syndrome, (14) and Van der Woude syndrome (15) taurodontism has also been found. Several theories of pathogenesis of such anamoly have also been proposed: an unusual developmental pattern, a delay in pulpal chamber calcification, Hertwig's epithelial root sheath (HERS) alteration, an odontoblastic deficiency.…”

Section: Introductionmentioning

confidence: 99%

Taurodontism: An anatomical challenge to clinical endodontics

Baranwal¹

2016

APRD

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While considering the various developmental anomalies of tooth, taurodontism is one of the morpho-anatomical anomalies which is rarely seen. The lack of constriction at the level of cemento-enamel junction with elongated pulp chambers and apical shift of bifurcation or trifurcation of roots, gives the tooth a rectangular or cylindrical appearance. The root canal system complexity, changed configuration and sometimes, the presence of additional root canals offer a big clinical challenge while performing the endodontic therapy. Till date, most of dental practitioners are having little awareness about such condition and its endodontic management.Therefore, this paper reviews about the details of taurodontism including its etiology, pathogenesis, diagnosis, types, categorization and difficulties produced during endodontic management along with their solutions.

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Dental characteristics of fibrous dysplasia and McCune-Albright syndrome

Cited by 82 publications

References 21 publications

Monostotic fibrous dysplasia: a case report with cone-beam computed tomography findings

Monostotic fibrous dysplasia: a case report with cone-beam computed tomography findings

Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Taurodontism: An anatomical challenge to clinical endodontics

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