Giancotti A, Romanini G, Di Girolamo R, Arcuri C
Department of Orthodontics, Ospedale, Fatebenefratelli-Isola, Tiberina, Rome, Italy.
Orthod Craniofac Res. 2002 Feb;5(1):59-63. doi: 10.1034/j.1600-0544.2002.01165.x.
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.
贝克威思-维德曼综合征(BWS)是一种罕见的遗传性疾病,与11号染色体短臂的改变有关,包括多种先天性异常。巨舌是主要表现,随后牙槽结构突出,导致下颌前突、前牙开牙合、下颌角异常钝角和下颌长度增加。本文介绍了一名早期进行舌部缩小治疗的患者使用矫形器进行的微创治疗。这项工作总结了与巨舌相关的口腔体征,并强调了巨舌对下颌生长结构的影响。我们认为,舌切除术可以在儿科患者中作为一种预防措施进行,因为它可以抑制舌头对骨骼生长的影响,并显著缩短后续治疗的持续时间和范围。