Rubinstein-Taybi syndrome: principal oral and dental disorders and literature update

Alejandro Carlos de la Parte-Serna, Ricardo Ortega-Soria, G. Oliván-Gonzalvo
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Abstract

Introduction: Oral and dental (OD) disorders in children with Rubinstein-Taybi syndrome (RTS) are frequent but not well-known by dentists and pediatricians due to the syndrome being extremely rare. Objective: To describe the OD findings observed in a 5-year-old girl with RTS and to update the literature. Clinical case: The patient presented the following OD manifestations: prominent lower lip, narrow mouth opening, narrow and arched palate, history of angular cheilitis, micrognathia, poor lingual motility, plaque and tartar, bleeding from gingival areas due to poor dental prophylaxis, and malocclusion in the form of an anterior open bite. These OD manifestations are seen in more than 40-60% of patients with RTS. Conclusions: Professionals who treat children with RTS should become aware of the advisability of referring them to the pediatric dentist from 1 year of age and performing check-ups every 6 months. Dental management is often difficult so collaboration with anesthesiologists is recommended in order to carry out a safe and effective treatment.
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鲁宾斯坦-泰比综合征:主要口腔和牙齿疾病及文献更新
鲁宾斯坦-泰比综合征(RTS)儿童的口腔和牙科(OD)疾病是常见的,但由于该综合征极为罕见,牙医和儿科医生并不了解。目的:描述一名5岁RTS女孩的OD发现并更新文献。临床病例:患者表现为:下唇突出,开口狭窄,上颚狭窄拱起,有角状口唇炎病史,小颌,舌动力差,牙菌斑和牙垢,牙齿预防不良导致牙龈出血,前牙开咬错。超过40-60%的RTS患者出现这些OD表现。结论:治疗RTS儿童的专业人员应该意识到从1岁开始将儿童转介给儿科牙医并每6个月进行一次检查的可取性。牙科管理通常是困难的,因此建议与麻醉师合作,以便进行安全有效的治疗。
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