Binder综合征的病因分析与治疗策略

The etiological analysis and treatment strategies of Binder syndrome

Binder综合征,又称为上颌-鼻发育不良,是一种罕见的先天性发育异常,目前对其病因和发病机制的认识尚不清楚,也缺少规范、公认的治疗策略指导。作者对Binder综合征病因和治疗方式的相关研究进行了回顾和分析,总结如下:(1)已知的Binder综合征病因分为遗传和非遗传两类,其核心为维生素K依赖性蛋白质活性的丧失。(2)治疗方式包括正畸治疗、鼻整形术和正颌手术。其中正畸治疗可以在任何阶段进行,而鼻整形术与正颌手术应被推迟至中面部发育完成后进行,理想的时机分别为14岁后和16~18岁后;鼻整形术的首选移植材料为自体肋软骨,合成材料是肋软骨不足或不能被获取时的备用选择;上颌截骨术主要适用于伴有安氏Ⅲ类错颌畸形的重度患者的治疗。

Binder syndrome,also known as maxillonasal dysplasia,is an uncommon developmental anomaly.The etiology and pathogenesis of Binder syndrome are still poorly understood,and there is a lack of normative consensus on its treatment strategies.This review retrospectively analyzed relevant studies on the etiology and treatment of Binder syndrome.The main points are summarized as follows:(1)Taken together,the etiology of Binder syndrome can be classified as genetic and non-genetic,whose crucial factor is the inactivation of vitamin K-dependent protein.(2)The management consists of orthodontic treatment,rhinoplasty,and orthognathic surgery.Orthodontic treatment can be performed at any time,while rhinoplasty and orthognathic surgery should be deferred until midfacial growth is almost complete.Therefore,the ideal timing of rhinoplasty is after 14 years old,and for orthognathic surgery,it is after 16-18 years old.Autologous costal cartilage is preferred for rhinoplasty and alloplastic materials are alternatives when donor availability is limited.Maxillary osteotomies should be reserved only for severe patients with Angle ClassⅢmalocclusion.