Defabianis Patrizia, Ninivaggi Rossella, Romano Federica
Department of Surgical Sciences, C.I.R. Dental School, University of Turin, 10126 Turin, Italy.
J Clin Med. 2022 Sep 26;11(19):5685. doi: 10.3390/jcm11195685.
Due to associated maxillofacial growth anomalies and the impairment of oral functions, macroglossia may negatively impact the oral health-related quality of life (OHRQoL) of people with Beckwith-Wiedemann syndrome (BWS). Therefore, the aim of this cross-sectional study was to determine the OHRQoL of Italian children and adolescents with BWS compared to healthy peers and to identify which symptoms related to macroglossia had the highest impact. A total of 48 patients with BWS and 48 age- and gender-matched controls completed the Italian version of OHIP-14 and a questionnaire on functional, oral and aesthetic outcomes. Parents of patients with BWS who had undergone tongue reduction surgery (TRS) answered additional questions related to surgery. The BWS group scored higher than controls on the total OHIP-14 and on the dimensions of oral function ( 0.036) and psychosocial impact ( 0.002), indicating a reduced OHRQoL. Neither gender nor age had an impact on OHRQoL. Scores were worse in children and adolescents treated with TRS, as most of them still had open bite malocclusion and speech difficulties. The OHRQoL of children and adolescents affected by BWS is worse than that of their healthy peers in spite of the surgical treatment of macroglossia.
由于相关的颌面生长异常和口腔功能受损，巨舌症可能会对患有贝克威思-维德曼综合征（BWS）的人的口腔健康相关生活质量（OHRQoL）产生负面影响。因此，这项横断面研究的目的是确定与健康同龄人相比，意大利患有BWS的儿童和青少年的OHRQoL，并确定与巨舌症相关的哪些症状影响最大。共有48名BWS患者和48名年龄和性别匹配的对照组完成了意大利版的OHIP-14以及一份关于功能、口腔和美学结果的问卷。接受过舌减容手术（TRS）的BWS患者的父母回答了与手术相关的其他问题。BWS组在OHIP-14总分以及口腔功能维度（0.036）和心理社会影响维度（0.002）上的得分高于对照组，表明OHRQoL降低。性别和年龄对OHRQoL均无影响。接受TRS治疗的儿童和青少年得分更差，因为他们中的大多数仍有开牙合错牙合和言语困难。尽管对巨舌症进行了手术治疗，但受BWS影响的儿童和青少年的OHRQoL仍比他们健康的同龄人差。
