THE "ELFIN FACE": CRANIOFACIAL AND DENTAL ASPECTS OF THE WILLIAMS-BEUREN SYNDROME
Por:
Maurino, V, Azzi, L, Vinci, R, Croveri, F, Boggio, A, Silvestre-Rangil, J, Tettamanti, L and Tagliabue, A
Publicada:
1 abr 2017
Resumen:
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.
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