A less-invasive approach with orthodontic treatment in Beckwith-Wiedemann patients.
The Beckwith-Wiedemann syndrome (BWS) is a rare genetic disorder, linked to an alteration on the short arm of chromosome 11 that comprises multiple congenital anomalies. Macroglossia is the predominant finding, with subsequent protrusion of dentoalveolar structures, which results in a protruding mandible, anterior open bite, abnormally obtuse gonial angle and increased mandibular length. A less-invasive treatment with orthopaedic appliances in a patient with early tongue reduction is presented. This work summarizes the oral signs linked to macroglossia, and highlights the influence of macroglossia on mandibular growth structures. In our opinion, glossotomy could be carried out in the paediatric patient as a preventive measure in that it curbs the tongue's influence on skeletal growth and dramatically reduces the duration and extensiveness of subsequent treatment.
Giancotti
A
A
Department of Orthodontics, Ospedale, Fatebenefratelli-Isola, Tiberina, Rome, Italy. giancotti@uniroma2.it
Romanini
G
G
Di Girolamo
R
R
Arcuri
C
C
D000367:Age Factors; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications* / Q000235:genetics / Q000628:therapy*; D002880:Chromosomes, Human, Pair 11; D005260:Female; D005927:Glossectomy; D006801:Humans; D007231:Infant, Newborn; D008260:Macroglossia / Q000150:complications / Q000209:etiology / Q000601:surgery*; D008313:Malocclusion, Angle Class III / Q000209:etiology / Q000628:therapy*; D020247:Myofunctional Therapy; D024343:Open Bite / Q000209:etiology / Q000628:therapy*; D017289:Orthodontic Appliances, Functional; D009971:Orthodontics, Corrective / Q000379:methods