先天性小耳畸形及其伴发畸形多中心临床研究  

Multicentric clinical study of clinical phenotypic analysis of microtia

作　　者：刘柳[1] 赵雪莲[2] 王海涛[3] 李高峰[4] 李正勇[5] 潘博 杨磊[1] Liu Liu;Zhao Xuelian;Wang Haitao;Li Gaofeng;Li Zhengyong;Pan Bo;Yang Lei(Department of Plastic Surgery,Third Hospital of Hebei Medical University,Shijiazhuang 050051,China;Department of Plastic Surgery,Second Hospital of Hebei Medical University,Shijiazhuang 050000,China;Department of Plastic Surgery,Weihai Municipal Hospital,Weihai 264200,China;Department of Plastic Surgery,Hunan Provincial People′s Hospital,Changsha 410005,China;Department of Plastic Surgery,West China Hospital,Sichuan University,Chengdu 610041,China;Seventh Department of Plastic Surgery Hospital,Chinese Academy of Medical Sciences,Peking Union Medical College,Beijing 100144,China)

机构地区：[1]河北医科大学第三医院整形外科,石家庄050051 [2]河北医科大学第二医院整形外科,石家庄050000 [3]山东大学威海市立医院整形外科,264200 [4]湖南省人民医院整形激光美容科,长沙410005 [5]四川大学华西医院整形外科,成都610041 [6]中国医学科学院北京协和医学院整形外科医院整形七科,100144

出　　处：《中华整形外科杂志》2020年第1期13-19,共7页Chinese Journal of Plastic Surgery

基　　金：国家自然科学基金(81571863,81871574);公益性科研院所基本科研业务费专项资金资助(2018PT31051)。

摘　　要：目的研究小耳畸形患者伴发其他畸形的特点,并探讨两者之间是否存在关联。方法收集2016年1月至2017年12月河北医科大学第三医院、河北医科大学第二医院、山东大学威海市立医院、湖南省人民医院、四川大学华西医院、中国医学科学院整形外科医院6家三甲医院收治的小耳畸形患者资料,通过体格检查、心电图、胸部CT和腹部超声检查进行诊断,记录并统计耳部畸形及伴发其他系统畸形的情况。采用SPSS 19.0统计学软件录入数据并处理,Pearsonχ^2检验分析小耳畸形与伴发畸形间的关系,以P<0.05为差异有统计学意义。结果共纳入小耳畸形患者746例,男性513例,女性233例,年龄5~27岁。单侧小耳678例,双侧小耳68例;Ⅰ度128例,Ⅱ度564例,Ⅲ度54例。336例(45%)患者伴发其他畸形,其中伴发耳面颈畸形321例,肌肉骨骼系统畸形261例,心血管系统畸形76例,泌尿生殖系统畸形30例,眼畸形30例,呼吸系统畸形27例,中枢神经系统畸形7例,消化系统畸形5例,膈疝1例。男性和女性小耳畸形伴发其他畸形的构成比分别为47.0%(241/513)和40.8%(95/233),两者比较差异无统计学意义(χ^2=2.493,P=0.114);单侧和双侧小耳畸形伴发其他畸形构成比分别为45.7%(310/678)和38.2%(26/68),两者比较差异无统计学意义(χ^2=1.400,P=0.237);Ⅰ、Ⅱ、Ⅲ度伴发畸形的构成比分别为39.8%(51/128)、42.6%(240/564)和83.3%(45/54),三者比较差异有统计学意义(χ^2=34.794,P<0.001)。结论小耳畸形具有显著的临床异质性,伴发畸形比例较高,耳廓发育程度越差的患者伴发畸形的几率越大;需要进行系统检查和辅助检查,有助于临床诊疗和致病原因分析。Objective This study collected and analyzed data from patients with microtia in multiple clinical centers to obtain the incidence characteristics of microtia in Chinese.Methods Data from 746 patients with microtia were collected from six hospitals,including theThird Hospital of Hebei Medical University,Hunan Provincial People′s Hospital,the Second Hospital of Hebei Medical University,Weihai Municipal Hospital,West China Hospital Sichuan University,Plastic Surgery Hospital,Chinese Academy of Medical Sciences from January 2016 to December 2017.These data included the results of physical examination and the results of auxiliary examination such as electrocardiogram,chest CT,and abdominal ultrasound.Then,the accompanying malformations of the microtia were statistically analyzed and discussed.Data were entered and processed using SPSS 19.0 statistical software.The relationship between microtia deformities and associated malformations was analyzed by Pearsonχ^2 test.P<0.05 was considered statistically significant.Results A total of 746 patients with microtia were included,including 513 males and 233 females,aged 5-27 years old.There were 678 cases of unilateral microtia,68 cases of bilateral microtia;128 cases ofⅠdegree,564 cases ofⅡdegree,and 54 cases ofⅢdegree.In addition,336 cases(45%)were associated with dysplasia of other systems.Detected anomalies were 321 cases of the ear,face,and neck,261 cases of musculoskeletal system and 76 cases of cardiovascular system,30 cases of anomalies of the urogenital system,eye,27 cases of respiratory system,7 cases of central nervous system,5 case of digestive system and 1case of diaphragmatic hernia.The proportion of male and female with microtia was 47.0%(241/513)and 40.8%(95/233),respectively,and there was no significant difference in between(χ^2=2.493,P=0.114);The composition ratios of unilateral and bilateral associated malformations were 45.7%(310/678)and 38.2%(26/68),and there was no significant difference in between(χ^2=1.400,P=0.237);The composition ratios of�

关 键 词：先天性小耳畸形 伴发畸形 多中心临床研究 

分 类 号：R76[医药卫生—耳鼻咽喉科]