Orthodontic Management of Children with Beckwith-Wiedemann Syndrome: An Evidence-based Review

Abstract

Background: Patients with Beckwith-Wiedemann syndrome (BWS) often present with macroglossia and aberrant dentoskeletal features, which may increase the prevalence of malocclusion and complicate conventional orthodontic management (OM). The aim was to comprehensively review the available literature regarding the OM of patients with BWS. Materials and Methods: Indexed databases were searched until February 2023. Clinical studies that addressed the dentoskeletal manifestations and OM of patients with BWS were included. A literature search was performed in accordance with the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analysis. Results: Notably, 8 out of 2,664 initially-identified studies were included. Six of the studies were case reports/case series and two were retrospective clinical studies. A total of 35 patients with BWS who received orthodontic treatment (OT) were included. Common dentoskeletal and soft tissue traits included the presence of macroglossia, obtuse gonial angle, and anterior open bite. Some form of maxillary transverse discrepancy was found in nine patients. The OT modalities that were utilized included extraoral appliances, functional appliances, removable appliances, interarch elastics, and fixed appliances. The outcome of the OT was reported as successful in five studies. The follow-up, as reported by three studies, ranged from 8 months to 6 years, and the results of the OT were maintained. Adjunct interdisciplinary therapeutic procedures included glossectomy, oral physiotherapy, and orthognathic surgery. Conclusions: Based upon limited available evidence, OT in conjunction with tongue reduction surgery and/or myofunctional therapy can be successfully performed in children with BWS to manage various dentoskeletal discrepancies.