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.
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References
Jorgenson RJ, Levin LS, Cross HE, Yoder F, Kelly TE. The Rieger syndrome. Am J Med Genet 1978;2: 307–318.
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.
Riise R, Storhaug K, Brøndum-Nielsen K. Rieger syndrome is associated with PAX6 deletion. Acta Ophthalmol Scand 2001;79: 201–203.
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.
Winter R, Baraitser M. London Dysmorphology Database. Oxford Uk. Oxford Medical Press, 1996.
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Skogedal, N., Nordgarden, H. Case Report: Rieger Syndrome. Eur Arch Paediatr Dent 8 (Suppl 1), 39–41 (2007). https://doi.org/10.1007/BF03262609
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DOI: https://doi.org/10.1007/BF03262609