第一、二鳃弓综合征诊疗进展  

The advance of the first and second branchial arch syndrome

作　　者：付菲 丁明超 田磊 刘彦普 

机构地区：[1]解放军第一八一医院,桂林541002 [2]空军军医大学第三附属医院,西安710032

出　　处：《中华口腔医学研究杂志（电子版）》2018年第2期126-130,共5页Chinese Journal of Stomatological Research(Electronic Edition)

摘　　要：第一、二鳃弓综合征的发病率在颌面部发育不全中位列第二,仅次于唇腭裂。它的发病机制并不明确,目前主要有基因学说、中胚层发育缺陷、镫骨动脉发育缺陷学说等,多数学者倾向于镫骨动脉发育缺陷学说。其临床表现包括下颌骨发育不全、眼眶、耳、神经、肌肉、软组织异常等,甚至还包括颌面部以外的发育不全。其表型差异较大,目前对于第一、二鳃弓综合征的临床诊断并没有一个标准,多依靠临床表现进行诊断。它的综合分型多采用OMENS分型法,近来又增加一些改良的OMENS+分型。第一、二鳃弓综合征的治疗需要多学科综合序列治疗。本文概述第一、二鳃弓综合征的发病机制、临床表现、分型、诊断与鉴别诊断、临床治疗方法,为该疾病的治疗提供帮助。The first and second branchial arch syndrome(HFM)is one of the most common congenital anomaly treated in craniofacial center worldwide,second to the cleft lip and palate. The pathogenesis of the HFM remains unclear. Popular hypothesis for the pathogenesis of the HFM include gene defection,mesoderm defection and stapes artery disruption. The syndrome encapsulates congenital anomalies of mandible,orbit,the external and middle ear,facial and trigeminal nerves,surrounding soft tissues on the affected side,and extracranial manifestations. Clinical manifestations of patients vary widely. Diagnostic criteria for HFM does not exist current,and the HFM diagnosis is based on physical exam findings of hypoplasia,aplasia,or malformation of the congenital or extracranial manifestations. The most widely used classification system is the OMENS classification scheme,and which later has been modified as the OMENS+ includes extracranial manifestations. A comprehensive sequential therapy with multidisciplinary coordination is needed to optimize the treatment for HFM. We reviewed the etiology,clinical characteristics,classification,diagnosis,surgical treatments for HFM.

关 键 词：第一、二鳃弓综合征 牵张成骨 骨移植 OMENS分型 综合序列治疗 

分 类 号：R782.2[医药卫生—口腔医学]