Dental and craniofacial anomalies of Axenfeld‐Rieger syndrome

Academic Article


  • Apart from the three distinguishing findings for the diagnosis of Axenfeld‐Rieger syndrome (oligodontia, failure of periumbilical skin to involute, and ocular anterior chamber defects), a common feature is midfacc hypoplasia. Two theories have been proposed to explain the midfacc hypoplasia. One theory suggests a pleiotropic gene effect of defective neural crest cells, while the other theory proposes that midface hypoplasia is a local effect due to the absence of teeth. This study presents an evaluation of the craniofacial defects in a family affected with Axenfeld‐Rieger syndrome. The maxillary deficiency in three affected patients was not limited to the alveolar regions. We conclude that a combination of skeletal and dentoalveolar factors contributes to the midface hypoplasia associated with Axenfeld‐Rieger syndrome. Copyright © 1986, Wiley Blackwell. All rights reserved
  • Authors

    Digital Object Identifier (doi)

    Author List

  • Childers NK; Wright JT
  • Start Page

  • 534
  • End Page

  • 539
  • Volume

  • 15
  • Issue

  • 10