目的:EEC是一种罕见的综合征,其特征是三联征。外胚层发育不良,和口面裂开,以及其他主要在头发上的临床表现,皮肤,和牙齿。本文旨在进行范围审查,以收集最相关的研究,并着重于儿科牙科环境中EEC综合征的诊断和口腔管理。这篇评论还假装提出建议并绘制了该临床主题的差距。
方法:在四个数据库中进行了详尽的电子和手动搜索(PubMed,EMBASE,谷歌学者,和牙科和口腔科学来源/EBSCO)根据先前建立的资格标准,使用不同的关键字组合,MeSH术语,和布尔运算符。标题,摘要,和全文文章由预先校准的审稿人筛选和选择。还完成了数据图表,以总结证据的概述。
结果:共确定了37个参考,删除重复项后,剩下32个标题;然后,仔细审查了25篇潜在的全文文章。最后,包括15项相关且信息量最大的研究。大多数研究是单临床病例报告。仅检测到一项描述性回顾性研究。没有发现随机临床试验或比较观察性研究。EEC综合征的管理需要医学/牙科多学科方法。
结论:必须涉及各种牙科专家。儿科牙医必须在预防和治疗口腔疾病方面发挥主要作用;特别是保护主要牙列和混合牙列,试图实现正常的口面生长。
OBJECTIVE: EEC is a rare syndrome characterized by the triad of ectrodactyly, ectodermal dysplasia, and orofacial clefting, along with other clinical manifestations mainly in hair, skin, and teeth. The present paper aimed to perform a scoping review to collect the most relevant studies and focused on the diagnosis and oral management of EEC syndrome in the pediatric dental setting. This review also pretended to make recommendations and map the gaps in this clinical topic.
METHODS: An exhaustive electronic and manual search was conducted in four databases (PubMed, EMBASE, Google Scholar, and Dentistry & Oral Sciences Source/EBSCO) according to previously established eligibility criteria, using different combinations of keywords, MeSH terms, and Boolean operators. Titles, abstracts, and full-text articles were screened and selected by precalibrated reviewers. A data charting was also accomplished for summarizing the overview of the evidence.
RESULTS: A total of 37 references were identified, and 32 titles remained after removing duplicates; then, 25 potential full-text articles were carefully reviewed. Finally, 15 relevant and most informative studies were included. Most studies were single clinical case reports. Only one descriptive retrospective study was detected. None randomized clinical trials or comparative observational studies were found. A medical/dental multidisciplinary approach is needed for the management of EEC syndrome.
CONCLUSIONS: Diverse dental specialists must be involved. Pediatric dentists must play a principal role in the prevention and treatment of oral diseases; particularly the preservation of the primary and mixed dentitions, trying to achieve normal orofacial growth.