先天性外中耳畸形标准化诊疗体系建设

Standardized diagnosis and treatment system construction for congenital outer and middle ear malformations

先天性外中耳畸形是头面部最常见的出生缺陷,包括耳廓形态畸形和结构畸形。随着诊疗新技术的快速发展,先天性外中耳畸形的标准化诊疗体系也在逐步建设完善。笔者团队提出新的综合性耳廓畸形分类、分型及分度方案。该方案有助于准确描述、定义耳廓畸形的形态特征,并指导每一特定类型畸形的治疗,对推动耳廓畸形的标准化诊治具有重要价值。耳廓畸形的常用治疗方式包括耳模矫正、耳整形和耳再造。为规范应用耳模矫正技术,组织撰写发布《先天性耳廓畸形耳模矫正技术专家共识》;对耳整形手术技术进行归类分析,核心在于解决皮肤重分配、软骨塑形和耳廓大小三类问题;为规范耳再造与听觉重建技术,牵头完成跨学科的《先天性外中耳畸形临床处理策略专家共识》和《先天性外中耳畸形及功能性耳再造国际共识建议》。外耳道狭窄是功能性耳再造技术应用的理想适应证,该类患者长期随访显示耳廓外形、外耳道健康程度、术后听力结果满意的比例高。通过目前优化的闭锁外耳道再造技术,术后外耳道健康情况较前期报道结果显著改善。团队还在先天性外中耳畸形标准化诊疗体系建设方面开展了系列研究。本文就研究成果及未来展望进行介绍。

Congenital outer and middle ear malformations are the most common birth defects,including morphological deformities and structural malformations.With the rapid development of diagnosis and treatment,the standardized diagnosis and treatment system for congenital outer and middle ear malformations have been gradually built and improved.Our team propose a new comprehensive classification system for auricular malformation,which helps to accurately describe the morphological features of outer ear,guide the treatment of each specific type,and promote the standardized diagnosis and treatment of auricular malformation.Regular treatments for ear malformations include ear molding,otoplasty surgery,and ear reconstruction.In order to standardize the application of ear molding technology,our team lead the consensus of Expert consensus on ear molding for congenital auricular deformation.We classify and analyze the otoplasty techniques,the core is to solve skin redistribution,cartilage shaping and auricle size.In order to standardize ear reconstruction,we lead the consensus of Clinical strategies for congenital outer and middle ear malformations and International consensus recommendations on microtia,aural atresia and functional ear reconstruction.Congenital aural stenosis is ideal indication for the application of functional ear reconstruction technology.The patients showed high proportion of satisfactory results with long term follow-up.Congenital aural atresia reconstruction had three major clinical problems.The health of the reconstructed ear canal was improved through the current optimized reconstruction technology compared with previous studies.Our team had carried out lots of research on the construction of standardized diagnosis and treatment system for congenital outer and middle ear malformations.This article introduced the construction achievements and future prospects.