Diseases & Conditions


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Dentin Dysplasia, Coronal


Synonyms of Dentin Dysplasia, Coronal
  • Anomalous Dysplasia of Dentin
  • Coronal Dentin Dysplasia
  • Dentin Dysplasia, Type II
  • Pulpal Dysplasia
  • Pulp Stones

Disorder Subdivisions



    General Discussion
    Coronal dentin dysplasia is a rare inherited dental defect that is also known as dentin dysplasia, type II. It is characterized by abnormal development (dysplasia) of the hard tissue (i.e., dentin) that is beneath the enamel, surrounds the pulp, and forms the major part of the teeth. In those with coronal dentin dysplasia, the baby teeth (primary or deciduous teeth) are brownish blue with a translucent opalescence. (Opalescence refers to a milky, opal-like display of colors in reflected light [iridescence].) However, the permanent teeth appear normal in color.

    As seen on dental x-ray imaging, the pulp chambers of the primary teeth--or the natural cavities that contain living pulp in the exposed portion of the teeth (crowns)--are obliterated by abnormal dentin. In addition, the pulp-containing canals within the roots of the teeth (root canals) are smaller than normal. The permanent teeth also have distinctive abnormalities of the pulp chambers. Coronal dentin dysplasia is transmitted as an autosomal dominant trait.
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    Organizations related to Dentin Dysplasia, Coronal
    • Genetic and Rare Diseases (GARD) Information Center
      PO Box 8126
      Gaithersburg MD 20898-8126
      Phone #: 301-519-3194
      800 #: 888-205-2311
      e-mail: gardinfo@nih.gov
      Home page: http://www.genome.gov/10000409
    • NIH/National Oral Health Information Clearinghouse
      1 NOHIC Way
      Bethesda MD 20892-3500
      Phone #: 301-402-7364
      800 #: --
      e-mail: nohic@nidcr.nih.gov
      Home page: http://www.nohic.nidcr.nih.gov



    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html