Williams Syndrome is a rare congenital disorder characterized by supravalvular aortic stenosis, peripheral pulmonary artery stenosis, mental retard and dysmorfic facial features. As regards the dental aspects of the syndrome, the deletion of the elastin gene induced clinicians to suspect periodontal alterations with a greater frequency of gingivo-periodontitis, but on the contrary no association between the syndrome and periodontal diseases have been found. Furthermore, patients show a higher frequency of teeth hypoplasia, an abnormal tooth morphology during primary dentition (12.5%) and during permanent dentition. We present a case report of a 12-year-old Caucasian boy affected by Williams-Beuren Syndrome who visited our hospital for a dental and orthodontic evaluation.

The "Elfin Face": Craniofacial & dental aspects of the williams-beuren syndrome

Maurino, V.;Azzi, L.;Croveri, F.;Boggio, A.;Tettamanti, L.;Tagliabue, A.
2017-01-01

Abstract

Williams Syndrome is a rare congenital disorder characterized by supravalvular aortic stenosis, peripheral pulmonary artery stenosis, mental retard and dysmorfic facial features. As regards the dental aspects of the syndrome, the deletion of the elastin gene induced clinicians to suspect periodontal alterations with a greater frequency of gingivo-periodontitis, but on the contrary no association between the syndrome and periodontal diseases have been found. Furthermore, patients show a higher frequency of teeth hypoplasia, an abnormal tooth morphology during primary dentition (12.5%) and during permanent dentition. We present a case report of a 12-year-old Caucasian boy affected by Williams-Beuren Syndrome who visited our hospital for a dental and orthodontic evaluation.
2017
http://www.biolifesas.org/contentsJBRHA.htm
Face; Malformation; Occlusion; Syndrome; Tooth; Endocrinology, Diabetes and Metabolism; Immunology and Allergy; Physiology; Immunology; Oncology; Endocrinology; Physiology (medical); Cancer Research
Maurino, V.; Azzi, L.; Vinci, R.; Croveri, F.; Boggio, A.; Silvestre-Rangil, J.; Tettamanti, L.; Tagliabue, A.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11383/2070102
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