Turkiye Klinikleri Journal of Dental Sciences

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Multiple Unerupted and Supernumerary Teeth in a Patient with Williams-Beuren Syndrome: An Unusual Case
Williams-Beuren Sendromlu Bir Hastada Multipl Sürmemiş Sürnumere Dişler: Nadir Bir Olgu Sunumu
Mesude ÇİTİRa, Ayşe Zeynep ZENGİNa
aDepartment of Dentomaxillofacial Radiology, Ondokuz Mayıs University Faculty of Dentistry, Samsun, TURKEY
Turkiye Klinikleri J Dental Sci. 2019;25(3):371-4
doi: 10.5336/dentalsci.2018-64084
Article Language: EN
Full Text
ABSTRACT
 Williams-Beuren syndrome (WBS) is a genetic disorder, caused by a deletion of chromosome 7. WBS characterized by typical facies and personality, cardiovascular disease, connective tissue growth and endocrine abnormalities. In the oral cavity, malocclusion, micrognathism, hypodontia, enamel hypoplasia, microdontia are among the wide spectrum of abnormalities identified in this disease. In this study, orofacial features of a 14-year-old male patient with WBS were presented. This case, contrary the literature, had multiple supernumerary teeth. The literature was reviewed because of this rare condition.

Keywords: Congenital abnormalities; tooth abnormalities; Williams Beuren syndrome
ÖZET
 Williams Beuren sendromu (WBS) kromozom 7'de mikrodelesyonların neden olduğu genetik bir bozukluktur. WBS kardiyovasküler hastalık, karakteristik fasiyal ve kişilik özellikleri, bağ dokusu büyümesi ve endokrin anormallikleri ile karakterizedir. İntraoral olarak maloklüzyon, mikrognati, hipodonti, mine hipoplazisi ve mikrodonti tespit edilen geniş anomali yelpazesi arasındadır. Bu çalışmada, WBS'li 14 yaşındaki bir erkek hastanın orofasiyal özellikleri sunulmuştur. Bu olguda, literatürün aksine, multipl sürnumere dişler mevcuttur. Nadir görülen olgu hakkında literatür bilgileri yeniden gözden geçirilmiştir.

Anahtar Kelimeler: Diş anomalileri; doğumsal anomaliler; Williams Beuren sendromu
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