[Embryology of the face and oto-mandibular dysplasia].
Otomandibular dysplasias encompass a broad range of congenital malformations (hemifacial microsomia, mandibulofacial dysostosis) affecting both jaw and ear apparatus. Deciphering the mechanisms of normal embryonic development is a prerequisite for optimal clinical management of those malformations. The development of craniofacial structures is a multi-step process, which involves many developmental events ranging from the migration of neural crest cells from the neural primordium, the molecular interactions that coordinate outgrowth and patterning of the facial primordia, to the fine tuning of the skeletal components. Our knowledge concerning craniofacial development has been gain through experiments carried out in animal developmental models; cell tracing strategies and functional analyses have contributed to significantly increment our understanding of human otomandibular dysplasias. In this review, we discuss classical and recent aspects of otomandibular development. Current proposals for pathogenesis are reviewed and a clinical approach for mandibulofacial dysostosis is proposed.
Charrier
Jean-Baptiste
JB
Service de chirurgie de la face et du cou du Pr Bobin, CHU de Bicêtre, AP-HP, 78, avenue du Général Leclerc, 94275 Kremlin Bicêtre Cedex, France. jean-baptiste.charrier@bct.aphp.fr
Creuzet
Sophie
S
D000818:Animals; D019465:Craniofacial Abnormalities / Q000196:embryology* / Q000235:genetics; D004423:Ear / Q000002:abnormalities* / Q000196:embryology; D047108:Embryonic Development / Q000235:genetics / Q000502:physiology; D005145:Face / Q000196:embryology*; D018507:Gene Expression Regulation, Developmental / Q000235:genetics; D006801:Humans; D008334:Mandible / Q000002:abnormalities* / Q000196:embryology; D015398:Signal Transduction / Q000235:genetics / Q000502:physiology