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中国特色临床诊所中小耳畸形患者:临床异质性及相关先天性畸形。

Microtia in a Chinese Specialty Clinic Population: Clinical Heterogeneity and Associated Congenital Anomalies.

机构信息

From the Department of Auricular Reconstruction, Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College; and the Biomedical Research Center, Hunan University of Medicine.

出版信息

Plast Reconstr Surg. 2018 Dec;142(6):892e-903e. doi: 10.1097/PRS.0000000000005066.

Abstract

BACKGROUND

Microtia is a congenital anomaly of the external ear that can appear in isolation or in association with other congenital anomalies. In this study, the authors identify the prevalence and phenotypes of associated congenital malformations in patients with microtia in a Chinese specialty clinic population.

METHODS

Data were collected from 672 patients seen between December of 2014 and February of 2016 in the Department of Auricular Reconstruction at the Plastic Surgery Hospital of Peking Union Medical College. All patients were examined by trained clinicians and classified into one of three grades of microtia. Co-occurring congenital anomalies were detected and recorded.

RESULTS

The majority of study participants were male patients (72 percent), and most participants had unilateral microtia (93 percent, 68 percent of whom had right-side microtia). Two hundred ninety-three patients (44 percent) had one or more associated anomalies. The most commonly occurring comorbid malformations were those of the ear, face, and neck (40 percent of all associated malformations); musculoskeletal system (35 percent); and cardiovascular system (11 percent).

CONCLUSIONS

These data represent the first detailed and thematic study of microtia and associated congenital anomalies in a Chinese clinical population. Substantial clinical heterogeneity was observed, and the prevalence of comorbid congenital malformations was high. Future studies investigating congenital anomalies associated with microtia are needed to improve understanding of its cause.

摘要

背景

小耳畸形是一种外耳先天性畸形,可单独出现或与其他先天性畸形同时出现。在这项研究中,作者在中国某专科诊所人群中确定了小耳畸形患者伴发先天性畸形的患病率和表型。

方法

本研究数据来自于 2014 年 12 月至 2016 年 2 月期间在北京协和医学院整形外科医院耳再造科就诊的 672 例患者。所有患者均由经过培训的临床医生进行检查,并分为小耳畸形 3 个等级之一。同时记录伴发的先天性畸形。

结果

大多数研究参与者为男性(72%),大多数参与者为单侧小耳畸形(93%,其中 68%为右侧小耳畸形)。293 例(44%)患者存在一种或多种伴发畸形。最常见的合并畸形是耳、面部和颈部畸形(所有伴发畸形的 40%);骨骼肌肉系统畸形(35%);和心血管系统畸形(11%)。

结论

这些数据代表了在中国临床人群中小耳畸形及其伴发先天性畸形的首次详细和专题研究。观察到明显的临床异质性,伴发先天性畸形的患病率较高。需要进一步研究小耳畸形伴发的先天性畸形,以增进对其病因的了解。

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