Suppr超能文献

面侧裂畸形综述:对该疾病认识的最新进展。

Hemifacial Microsomia Review: Recent Advancements in Understanding the Disease.

机构信息

T. Marciniak Lower Silesian Specialist Hospital.

Wroclaw Medical University, Wroclaw.

出版信息

J Craniofac Surg. 2020 Nov/Dec;31(8):2123-2127. doi: 10.1097/SCS.0000000000006616.

Abstract

Hemifacial microsomia (HFM) is the second most common congenital disability of the face, with a prevalence of 1 in 3000 to 5600 live births. Although etiology is still not fully understood, including both genetics and environmental factors, the latest reports indicate the prominence of premature loss of the neural crest cells. What is more, a deficit of muscles of mastication, except the masseter, correlates in the pathomechanism of mandibular underdevelopment. Due to the significant phenotypic diversification, the typical picture of HFM cannot be determined. It may present as an esthetic concern-minor asymmetry with deformed auricle, and on the contrary, as microtia/anotia with conductive type hearing loss, hypoplastic mandible, and microphthalmia, impairing patient's daily activities. Referring to psychosocial problems, it has been proved that in population with HFM, there is a modestly elevated risk for behavior problems, social competence, and less acceptance. Over the years, more comprehensive methods of assessing the extent and severity of the HFM as the OMENS (+) classification have emerged. The authors like to summarize and present for plastic surgery resident and plastic surgeons the critical features of HFM, including the epidemiology, clinical presentation, pathogenesis, and innovative management reported in the current literature.

摘要

半面短小畸形(HFM)是面部第二常见的先天性残疾,每 3000 至 5600 例活产中就有 1 例。尽管病因尚未完全阐明,包括遗传和环境因素,但最新报告表明神经嵴细胞过早缺失的突出性。更重要的是,咀嚼肌(除咬肌外)的不足与下颌骨发育不全的发病机制有关。由于表型显著多样化,无法确定 HFM 的典型表现。它可能表现为轻度不对称的美容问题——耳廓畸形,或者相反,表现为小耳/无耳伴传导性听力损失、下颌骨发育不良和小眼球,从而影响患者的日常活动。关于心理社会问题,已经证明 HFM 患者的行为问题、社会适应能力和接受程度存在适度升高的风险。多年来,出现了更多全面的评估 HFM 程度和严重程度的方法,如 OMENS(+)分类。作者希望为整形外科住院医师和整形外科医生总结和介绍 HFM 的关键特征,包括目前文献中报道的流行病学、临床表现、发病机制和创新性管理。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验