背景:莫比乌斯综合征(MS)是一种罕见的,非进步,神经肌肉,涉及口腔颌面部的同源性疾病。本研究旨在描述MS患者的口腔和口外发现及其全面的牙科管理。
方法:在PubMed/MEDLINE中进行了数字搜索,Scopus,WebofScience,和谷歌学者,仅限于2000年1月1日至2023年4月2日的英语文章,遵循PRISMA指南。按照JBI指南评估研究的方法学质量。对总体结果进行了定性分析,口外和口内表现,考虑适当的牙科管理。
结果:纳入了23项研究,对124例MS患者进行分析。82%的MS患者年龄小于15岁。最常见的口外表现是眨眼和视觉问题(78,22%),上肢和下肢畸形(58,22%),双侧面瘫(12,90%),缺乏面部表情(12.09%),和单侧面瘫(6,45%)。另一方面,最常见的口腔表现是舌畸形(78,22%),小颌畸形(37,90%),唇性无能(36,29%),腭裂(22.87%),哥特式腭(16,12%),微小口腔(15,32%),前开口咬伤(15,32%),龋齿(8.87%),和牙周病(8,06%)。大多数MS患者接受儿科牙科治疗(60,86%),使用手术方法(56,52%),和正畸和矫形上颌(43,47%),其次是修复(39,13%),和牙周治疗(21,73%)。
结论:本系统综述表明,MS患者存在多种口腔和口外表现,根据口腔表现为每个患者计划和定制牙科治疗。这些治疗包括解决问题和口腔健康维护,结合最近的技术来管理和治疗MS患者。
BACKGROUND: Moebius syndrome (MS) is a rare, non-progressive, neuromuscular, congenic disease involving the oral maxillofacial region. The present study aimed to describe the oral and extraoral findings in MS patients and their comprehensive dental management.
METHODS: A digital search was carried out in PubMed/MEDLINE, Scopus, Web of Science, and Google Scholar, restricted to articles in English from Jan 01, 2000, to Apr 02, 2023, following PRISMA guidelines. The methodological quality of the studies was evaluated following the JBI guidelines. Qualitative analysis was carried out on the overall result, extraoral and intraoral manifestations, considering dental management as appropriate.
RESULTS: Twenty-three studies were included, and a total of 124 cases of patients with MS were analyzed. The 82% of patients with MS were younger than 15 years of age. The most frequent extraoral manifestations were blinking and visual problems (78,22%), malformations of the upper and lower limbs (58,22%), bilateral facial paralysis (12,90%), lack of facial expression (12.09%), and unilateral facial paralysis (6,45%). On the other hand, the most frequent oral manifestations were tongue deformities (78,22%), micrognathia (37,90%), labial incompetence (36,29%), cleft palate (22,87%), gothic palate (16,12%), microstomia (15,32%), anterior open bite (15,32%), dental caries (8,87%), and periodontal disease (8,06%). The majority of MS patients were treated by pediatric dentistry (60,86%), using a surgical approach (56,52%), and orthodontic and orthopedic maxillary (43,47%) followed by restorative (39,13%), and periodontal treatments (21,73%).
CONCLUSIONS: This systematic review demonstrates that patients with MS present a wide variety of oral and extraoral manifestations, for which dental treatments are planned and tailored to each patient in accordance with oral manifestations. These treatments encompass problem resolution and oral health maintenance, incorporating recent techniques in managing and treating patients with MS.