Orofacial Findings and Dental Management of a Child with Williams Syndrome
DOI:
https://doi.org/10.15448/http://dx.doi.org/10.15448/1980-6523.2016.1.17770Keywords:
Williams syndrome, Agenesis, ChildrenAbstract
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
Issue
Section
License
COPYRIGHT
The submission of originals to Odonto Ciência implies the transfer by the authors of the right for publication. Authors retain copyright and grant the journal right of first publication. If the authors wish to include the same data into another publication, they must cite Odonto Ciência as the site of original publication.
CREATIVE COMMONS LICENSE
As this journal is open access, the articles are allowed free use in scientific and educational applications, with citation of the source.
According to the type of Creative Commons License (CC-BY 4.0) adopted by Odonto Ciência, the user must respect the requirements below.
You are free to:
Share — copy and redistribute the material in any medium or format.
Adapt — remix, transform, and build upon the material for any purpose, even commercially.
However, only under the following terms:
Attribution — You must give appropriate credit, provide a link to the license, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests Odonto Ciência endorses you or your use.
No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.
Notices:
You do not have to comply with the license for elements of the material in the public domain or where your use is permitted by an applicable exception or limitation.
No warranties are given. The license may not give you all of the permissions necessary for your intended use. For example, other rights such as publicity, privacy, or moral rights may limit how you use the material.
For more details on the Creative Commons license, please follow the link in the footer of this website.