Title :

Dental and maxillofacial features of noonan syndrome: case series of ten patients

Author(s) :

Lutz, Jean-Christophe [Auteur]
Université de Strasbourg [UNISTRA]
Nanomédecine Régénérative [NanoRegMed]
Nicot, Romain [Auteur]
Advanced Drug Delivery Systems (ADDS) - U1008
Médicaments et biomatériaux à libération contrôlée: mécanismes et optimisation - Advanced Drug Delivery Systems - U 1008 [MBLC - ADDS]
Schlund, Matthias [Auteur]
Advanced Drug Delivery Systems (ADDS) - U1008
Schaefer, Elise [Auteur]
Centre Hospitalier Universitaire [Strasbourg] [CHU Strasbourg]
Laboratoire de Génétique Médicale [LGM]
Bornert, Fabien [Auteur]
Nanomédecine Régénérative [NanoRegMed]
Fioretti, Florence [Auteur]
Nanomédecine Régénérative [NanoRegMed]
Ferri, Joel [Auteur]
Advanced Drug Delivery Systems (ADDS) - U1008
Médicaments et biomatériaux à libération contrôlée: mécanismes et optimisation - Advanced Drug Delivery Systems - U 1008 [MBLC - ADDS]

Journal title :

Journal of cranio-maxillo-facial surgery . official publication of the European Association for Cranio-Maxillo-Facial Surgery

Abbreviated title :

J Craniomaxillofac Surg

Volume number :

48

Pages :

242-250

Publication date :

2020-02-06

ISSN :

1878-4119

English keyword(s) :

Noonan syndrome
Giant cells
Dentofacial deformities
Orthodontics
Orthognathic surgery
Maxillofacial abnormalities

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Noonan syndrome (NS) is a relatively common congenital multiple-anomaly syndrome, resembling Turner syndrome, but without chromosomal anomaly. Besides the unusual facies, the maxillofacial and dental features of patients ...
Show more >Noonan syndrome (NS) is a relatively common congenital multiple-anomaly syndrome, resembling Turner syndrome, but without chromosomal anomaly. Besides the unusual facies, the maxillofacial and dental features of patients with NS are not well-summarized in the literature. The aim of this study was to describe these features and propose specific treatment guidelines for practitioners involved in oral and maxillofacial care. A retrospective multicentric study was conducted of 14 patients who were referred for NS screening. In total, 10 patients were found to carry a mutation involved in NS or NS-related disorders. Fifty percent of the mutations affected PTPN11. All patients presented with the typical extraoral features, such as macrocephaly, hypertelorism, ptosis, triangular face shape and ear dystrophy. Intraoral manifestations, including malocclusion (maxillary transversal deficiency, crossbite, anterior open-bite and class II malocclusion), dental anomalies (delayed eruption, agenesis and dystrophy, odontoma) and radiologic jaw lesions were identified in five out of 10 patients. These findings were searched in a review of the literature to obtain a comprehensive description of oral and maxillofacial features in patients with NS. The proposed treatment guidelines emphasize frequent coagulation anomalies that need to be considered prior to surgery. Early dental assessment and yearly follow-up with oral prophylaxis are recommended. Orthodontics and orthognathic surgery are also of primary importance in the management of NS patients.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Inserm
Université de Lille

Collections :

• Advanced Drug Delivery Systems (ADDS) - U1008

Submission date :

2021-01-20T15:59:20Z
2024-02-19T10:00:59Z