Case Report: Rieger Syndrome

  • N. SkogedalEmail author
  • H. Nordgarden


Background: Rieger syndrome is a rare autosomal dominant condition with at least three genetic forms. The main symptoms are umbilical cord anomalies, malformations of the anterior chamber of the eye, agenesis of certain teeth, and a hypoplastic mid-face. Case reports: In this paper three cases of Rieger syndrome are presented, focusing in particular on dental and craniofacial findings. Treatment: Treatments have been individualized and include temporary dentures and restoration of primary teeth to preserve them until their successors erupt. Follow-up: Patients with Rieger syndrome should be followed according to an individualized plan, depending on the severity of dental symptoms and general caries risk. Conclusion: It is important that the dental team have knowledge about this syndrome, as ocular complications can be prevented if the diagnosis is made early.

Key words

Rieger syndrome hypodontia frenulum treatment 


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Copyright information

© European Archives of Paediatric Dentistry 2007

Authors and Affiliations

  1. 1.TAKO-centreLovisenberg Diakonale HospitalOsloNorway

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