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Developmental disorders of the dentition: An update

Authors

  • Ophir D. Klein,

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    • Ophir D. Klein, M.D., Ph.D. is Associate Professor in the Departments of Orofacial Sciences and Pediatrics, Chair of the Division of Craniofacial Anomalies, and Director of the Program in Craniofacial and Mesenchymal Biology at UCSF. Dr. Klein's research focuses in large part on understanding the processes underlying craniofacial and dental development. His laboratory uses mouse models to study the mechanisms responsible for the normal and abnormal development of teeth, facial skeleton, and other organs, as well as the regeneration of these organs.
  • Snehlata Oberoi,

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    • Snehlata Oberoi, D.D.S., M.D.S. is Associate Professor of Orthodontics with the UCSF Center for Craniofacial Anomalies, where she provides assessment and treatment for children with craniofacial disorders. Her research focuses on developing new methods to assess the outcomes of treatment for cleft lip, cleft palate, and other craniofacial anomalies. She also collaborates with the Center's medical geneticists on research seeking to identify genetic mutations and how they affect dental and facial defects in various craniofacial anomalies.
  • Ann Huysseune,

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    • Ann Huysseune, Ph.D. is Professor and Head of the Biology Department at Ghent University, Belgium. Her research interests are focused on the development, structure, and evolution of the skeleton, with particular attention to teeth. She uses various teleost fish and other non-mammalian species to study evo–devo aspects of skeletal tissues and skeletal elements. Current studies in her group focus on the dermal skeleton (including the teeth) and on the vertebral column.
  • Maria Hovorakova,

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    • Maria Hovorakova, Ph.D. is a researcher in the Laboratory of Odontogenesis, Department of Teratology at the Institute of Experimental Medicine at the Academy of Sciences CR, Prague, Czech Republic. She is currently working on tooth development in wild-type and mutant mice, with a focus on the role of rudiments in tooth development.
  • Miroslav Peterka,

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    • Miroslav Peterka, M.D., Ph.D. is Head of the Department of Teratology at the Institute of Experimental Medicine at the Academy of Sciences CR, Prague, Czech Republic. He is an Associate Professor at the 1st Medical Faculty, Charles University in Prague and a clinical teratologist involved in prevention of inborn defects at the Clinic of Plastic Surgery, 3rd Medical Faculty in Prague. His research interests are experimental and clinical teratology, as well as pathogenesis and epidemiology of developmental anomalies.
  • Renata Peterkova

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    • Renata Peterkova, M.D., Ph.D. is Head of the Laboratory of Odontogenesis, Department of Teratology at the Institute of Experimental Medicine at the Academy of Sciences CR, Prague, Czech Republic. Her focus is morphology, including embryology, histology, and anatomy, and her field of interest is the normal and pathological development of teeth and adjacent structures. During her research career, she has studied rudimentary structures during orofacial development. She is interested in their role during normal ontogeny, their involvement in the origin of developmental anomalies, and their evolutionary significance.

Correspondence to: Ophir D. Klein, M.D., Ph.D., Director, Program in Craniofacial and Mesenchymal Biology, UCSF, San Francisco, CA. E-mail: ophir.klein@ucsf.edu

Abstract

Dental anomalies are common congenital malformations that can occur either as isolated findings or as part of a syndrome. This review focuses on genetic causes of abnormal tooth development and the implications of these abnormalities for clinical care. As an introduction, we describe general insights into the genetics of tooth development obtained from mouse and zebrafish models. This is followed by a discussion of isolated as well as syndromic tooth agenesis, including Van der Woude syndrome (VWS), ectodermal dysplasias (EDs), oral-facial-digital (OFD) syndrome type I, Rieger syndrome, holoprosencephaly, and tooth anomalies associated with cleft lip and palate. Next, we review delayed formation and eruption of teeth, as well as abnormalities in tooth size, shape, and form. Finally, isolated and syndromic causes of supernumerary teeth are considered, including cleidocranial dysplasia and Gardner syndrome. © 2013 Wiley Periodicals, Inc.

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