Advertisement

Case Report: Rieger Syndrome

  • N. SkogedalEmail author
  • H. Nordgarden
Article

Abstract

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 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Jorgenson RJ, Levin LS, Cross HE, Yoder F, Kelly TE. The Rieger syndrome. Am J Med Genet 1978;2: 307–318.CrossRefPubMedGoogle Scholar
  2. Phillips JC, del Bono EA, Haines JL.et al. A second locus for Rieger syndrome maps to chromosome 13q14. Am J Hum Genet 1996;59: 613–619.PubMedGoogle Scholar
  3. Riise R, Storhaug K, Brøndum-Nielsen K. Rieger syndrome is associated with PAX6 deletion. Acta Ophthalmol Scand 2001;79: 201–203.CrossRefPubMedGoogle Scholar
  4. Semina EV, Reiter R, Leysens NJ, et al. Cloning and characterization of a novel bicoid-related homeobox transcription factor gene, Rieg, involved in Rieger syndrome. Nat Genet 1996;14: 392–399.CrossRefPubMedGoogle Scholar
  5. Winter R, Baraitser M. London Dysmorphology Database. Oxford Uk. Oxford Medical Press, 1996.Google Scholar

Copyright information

© European Archives of Paediatric Dentistry 2007

Authors and Affiliations

  1. 1.TAKO-centreLovisenberg Diakonale HospitalOsloNorway

Personalised recommendations