先天性小耳畸形患者颅颌面发育异常的研究进展

Review the research progress of congenital microtia patients with craniofacial dysplasia

先天性小耳畸形患者常伴发颅颌面发育异常,以不同程度的颅面骨骼、眼、面部软组织的发育不良为主,常见半侧颅面短小症和Treacher-Collins综合征。半侧颅面短小症下颌骨畸形突出,累及同侧上颌骨、颧骨、眼和软组织时可造成面部偏斜畸形,部分存在上呼吸道形态和功能异常,Treacher-Collins综合征面中部突出,眶颧发育不良,下颌后缩,颅颌面发育异常不仅造成患者外观毁损,相应器官的功能也会严重影响生活质量。影像和信息技术的发展促进了对小耳畸形患者的颅颌面结构的了解,但目前仍不清楚颅颌面发育异常的病理机制,现通过研究和分析文献,综述小耳畸形患者颅颌面发育异常特点的研究进展。

Patients with congenital microtia are often accompanied by craniofacial dysplasia,with different degrees of craniofacial skeleton,eye,and facial soft tissue dysplasia.Hemifacial microsomia and Treacher-Collins syndrome are most common syndromes.Hemifacial microsomia is characterised by mandibular deformity.Ipsilateral maxilla,zygomatic bone,eye,and soft tissue malformation can cause facial asymmestry,while some have abnormal upper airway morphology and function.Treacher-Collins syndrome is marked by midfacial protrusion,orbital-zygomatic dysplasia,mandibular retraction.craniofacial dysplasia not only cause the damaged appearance,but also affect the quality of life.The development of imaging and information technology has promoted the understanding of the craniofacial structure of patients with microtia,but the pathological mechanism is still unclear.Through research and analysis of literature,this paper reviews the characteristics of craniofacial dysplasia in patients with microtia.