Interdisciplinary dental care of patients with Williams–Beuren syndrome

Czech version

Authors: W. Urbanová ^1,2,3,4,5; J. Vašáková ^1,2,3,4,5; I. Dubovská ^6,7; P. Poláčková ^1,2,3,4,5; P. Křížová ^1,2,5; J. Borovec ^1,2,3,4,5*
Authors‘ workplace: Korespondující autor ^*; Stomatologická klinika, 3. lékařská fakulta, Univerzita Karlova, Praha ¹; Stomatologická klinika, Fakultní nemocnice Královské Vinohrady, Praha ²; Stomatologická klinika, Lékařská fakulta v Plzni, Univerzita Karlova ³; Stomatologická klinika, Fakultní nemocnice Plzeň ⁴; Rozštěpové centrum Praha při Fakultní nemocnici Královské Vinohrady ⁵; Klinika zubního lékařství, Lékařská fakulta Univerzity Palackého v Olomouci ⁶; Klinika zubního lékařství, Fakultní nemocnice Olomouc ⁷
Published in: Česká stomatologie / Praktické zubní lékařství, ročník 125, 2025, 4, s. 89-96
Category: Review Article
doi: https://doi.org/10.51479/cspzl.2025.008

Overview

Introduction and aim: Williams–Beuren syndrome (WBS) is characterized by developmental delay with varying degrees of intellectual disability, impaired elastin production affecting tissue elasticity throughout the body, distinctive facial dysmorphism, congenital cardiovascular defects, hyperacusis, and unique personality traits. The aim of this report is to familiarize members of the dental team with the specific challenges and management of these patients, based on information from the scientific literature.

Methodology: In both international and Czech scientific literature, all available articles on dental issues and craniofacial morphology in patients with WBS were retrieved from the databases PubMed, Scopus, Google Scholar, SciELO, and Prolékaře. The key words used included: “Williams–Beuren”, “craniofacial/kraniofaciální”, “teeth/zuby”, and “oral/orální”.

Results: A comprehensive search of both international and local scientific literature was conducted, identifying a total of 27 records, including two abstracts. Of these, four were original research studies focusing on dental issues, one was a review article, eight described the etiology, morphology, and development of craniofacial malformations, and twelve were case reports. Typical oral manifestations of WBS include morphological dental anomalies, agenesis of individual teeth, and orthodontic malocclusions affecting dental arches and jaw relationships. Patients also exhibit an increased incidence of dental caries and periodontal disease. Maintaining adequate oral hygiene can be challenging due to limited fine motor skills. Behavioural techniques should be preferred during examinations and treatment by general dentists, orthodontists, and dental hygienists, and background noise should be minimized. General anaesthesia poses a significant risk for patients with WBS; therefore, emphasis should be placed on preventing dental caries and its complications. Treatment planning should consider not only the intraoral findings but also the patient´s overall health and level of patient cooperation.

Conclusion: The care of patients with Williams–Beuren syndrome requires a thorough understanding of the condition by all members of the dental team, as well as adaptation to the unique needs and specific responses of these individuals. Further studies focusing on evidence-based dental recommendations are necessary.

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