Orofacial Findings and Dental Management of a Child with Williams Syndrome

Authors

  • Luciana Pereira Universidade Federal do Rio de Janeiro
  • Daniela Novaes Soares Universidade Federal do Rio de Janeiro
  • Rafael de Lima Pedro Universidade Federal do Rio de Janeiro
  • Tatiana Kelly da Silva Fidalgo Universidade Federal do Rio de Janeiro
  • Marcelo Castro Costa Universidade Federal do Rio de Janeiro
  • Gloria Fernanda Barbosa de Araújo Castro Universidade Federal do Rio de Janeiro

DOI:

https://doi.org/10.15448/http://dx.doi.org/10.15448/1980-6523.2016.1.17770

Keywords:

Williams syndrome, Agenesis, Children

Abstract

Williams syndrome (WS) is a multisystem, congenital and panethnic disorder characterized by developmental and physical abnormalities. Developmental abnormalities in dentition are frequently observed in various patients with craniofacial syndromes, however there are a few reports regarding dental management in patients with WS. The aim of the present report is to describe the case of a 6-year-old child with characteristic facial features of WS with remarkable orofacial findings and the ensuing dental management. An intraoral examination revealed a mixed dentition period, with tooth agenesis and caries requiring treatment such as extraction, restoration and fluoride application. The dental treatment was carried out under local anesthesia and was completed successfully. Since then clinical and radiographic evaluations have been conducted periodically. 

References

Williams JC, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation. 1961 Dec;241311-8.

Morris CA. Introduction: Williams syndrome. Am J Med Genet C Semin Med Genet. 2010 May 15;154C(2):203-8.

Stromme P, Bjornstad PG, Ramstad K. Prevalence estimation of Williams syndrome. J Child Neurol. 2002 Apr;17(4):269-71.

Kohase H, Wakita R, Doi S, Umino M. General anesthesia for dental treatment in a Williams syndrome patient with severe aortic and pulmonary valve stenosis: suspected episode of postoperatively malignant hyperthermia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Oct;104(4):e17-20.

Morris CA, Demsey SA, Leonard CO, Dilts C, Blackburn BL. Natural history of Williams syndrome: physical characteristics. J Pediatr. 1988 Aug;113(2):318-26.

Axelsson S, Bjornland T, Kjaer I, Heiberg A, Storhaug K. Dental characteristics in Williams syndrome: a clinical and radiographic evaluation. Acta Odontol Scand. 2003 Jun;61(3):129-36.

Lashkari A, Smith AK, Graham JM, Jr. Williams-Beuren syndrome: an update and review for the primary physician. Clin Pediatr (Phila). 1999 Apr;38(4):189-208.

Beuren AJ, Apitz J, Harmjanz D. Supravalvular aortic stenosis in association with mental retardation and a certain facial appearance. Circulation. 1962 Dec;261235-40.

Oncag A, Gunbay S, Parlar A. Williams syndrome. J Clin Pediatr Dent. 1995 Summer;19(4):301-4.

Moskovitz M, Brener D, Faibis S, Peretz B. Medical considerations in dental treatment of children with Williams syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 May;99(5):573-80.

American Academy of Pediatrics: Health care supervision for children with Williams syndrome. Pediatrics. 2001 May;107(5):1192-204.

Gosch A, Pankau R. Social-emotional and behavioral adjustment in children with Williams-Beuren syndrome. Am J Med Genet. 1994 Dec 1;53(4):335-9.

Downloads

Published

2016-11-17

Issue

Section

Case Report