van Nunen Daan P F, Kolodzynski Mischka N, van den Boogaard Marie-José H, Kon Moshe, Breugem Corstiaan C
Division of Plastic and Reconstructive Surgery; University of Utrecht Medical Center, Heidelberglaan 100, PO Box 85500, 3508 GA Utrecht, The Netherlands.
Division of Plastic and Reconstructive Surgery; University of Utrecht Medical Center, Heidelberglaan 100, PO Box 85500, 3508 GA Utrecht, The Netherlands.
Int J Pediatr Otorhinolaryngol. 2014 Jun;78(6):954-9. doi: 10.1016/j.ijporl.2014.03.024. Epub 2014 Mar 30.
In Europe there have been few detailed reports on the clinical characteristics of microtia patient populations. The objective of the present study is to contribute to our insight of microtia in Europe by examining the Dutch microtia population treated in the University Medical Center Utrecht (UMCU) with regards to its clinical features and associated anomalies. In addition, an overview of the literature is provided for thorough comparison.
A retrospective chart review was performed for all microtia patients referred to the UMCU for reconstructive surgery of the auricle over the period 1990-2012. Previous studies were identified by a systematic search of the electronic literature databases PubMed and Embase. In a subsequent meta-analysis the results from the literature review were pooled by geographical region to facilitate comparison.
A total of 204 microtia patients were referred for reconstructive surgery during 1990-2012. This group was characterized by a male predominance of 60.8%. Unilateral disease was observed in 91.7% of patients, affecting the right auricle in 66.3%. In unilateral patients lobule type microtia was seen in 59.9%, (small) concha type in 34.4% and anotia in 5.7%. The more frequent anomalies associated with microtia were atresia of the acoustic meatus (76.0%), preauricular skin tags (30.5%), hemifacial microsomia (27.5%), facial nerve paralysis (8.3%) and congenital heart disease (2.5%). Familial occurrence of microtia was reported for 2.0% of UMCU patients and for 10.0% of patients in the literature.
The clinical characteristics of microtia in the Netherlands correspond to those reported for other patient populations in the literature. Most congenital anomalies associated with microtia in Dutch patients belong to the Oculo-Auriculo-Vertebral Spectrum. The considerable degree of familial microtia observed in the literature points to a substantial genetic component in the etiology of the condition.
在欧洲，关于小耳畸形患者群体临床特征的详细报告较少。本研究的目的是通过研究在乌得勒支大学医学中心（UMCU）接受治疗的荷兰小耳畸形患者群体的临床特征和相关异常情况，增进我们对欧洲小耳畸形的了解。此外，还提供了文献综述以供全面比较。
对1990年至2012年期间转诊至UMCU进行耳廓重建手术的所有小耳畸形患者进行回顾性病历审查。通过系统检索电子文献数据库PubMed和Embase来识别先前的研究。在随后的荟萃分析中，按地理区域汇总文献综述的结果以方便比较。
1990年至2012年期间，共有204例小耳畸形患者转诊进行重建手术。该群体以男性为主，占60.8%。91.7%的患者为单侧病变，其中66.3%影响右耳。在单侧患者中，小叶型小耳畸形占59.9%，（小）耳甲型占34.4%，无耳畸形占5.7%。与小耳畸形相关的较常见异常包括外耳道闭锁（76.0%）、耳前皮肤赘生物（30.5%）、半侧颜面短小（27.5%）、面神经麻痹（8.3%）和先天性心脏病（2.5%）。UMCU患者中有2.0%报告有小耳畸形家族史，文献中该比例为10.0%。
荷兰小耳畸形的临床特征与文献中报道的其他患者群体的特征相符。荷兰患者中与小耳畸形相关的大多数先天性异常属于眼-耳-脊椎综合征。文献中观察到的相当程度的小耳畸形家族史表明该病病因中有很大的遗传成分。
