小儿小畸形的临床及影像学评价。

F Calzolari, G Garani, A Sensi, A Martini
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引用次数: 47

摘要

由于治疗问题的复杂性和父母的焦虑,先天性耳畸形儿童,特别是外耳严重受累的儿童的治疗是困难的。尽快制定一个完整的治疗/康复计划是非常重要的,即使外科手术推迟了。诊断成像在对儿童小耳畸形的整体评估中起着重要作用,以诊断可能相关的外耳道、中耳和内耳畸形。由于这些原因,我们对小耳畸形儿童的诊断方案包括耳科和听力学评估、临床遗传学和放射学成像,从新生儿时期开始。在这里,报告了27名完成诊断方案的儿童的数据。在27例病例中,有8例是双侧的,在单侧病例中,右侧的影响更频繁。41%的病例被诊断为其他先天性畸形。根据更大规模的文献研究,发现小耳症的程度与外耳和中耳发育不良的频率之间存在高度相关。内耳畸形发生率较低,但与小耳症程度无明显相关性。事实是,儿童与小耳症也可能有严重的内耳畸形强调。
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Clinical and radiological evaluation in children with microtia.

The management of a child with congenital ear malformation, in particular if the external ear is severely involved, is difficult because of the complexity of the therapeutic problem, and that of parental anxiety. It is very important to plan a complete therapeutic/habilitative programme as soon as possible, even if surgical procedures are delayed. Diagnostic imaging plays an important role in the global assessment of a child with microtia, in order to diagnose possible associated external auditory canal, middle and inner ear malformations. For these reasons our diagnostic protocol for children with microtia includes otological and audiological evaluation, clinical genetics and radiological imaging, from the neonatal period. Here, data are reported on 27 children with microtia who completed the diagnostic protocol. In eight of 27 cases microtia was bilateral: in unilateral cases the right side was affected more frequently. Other congenital malformations were diagnosed in 41% of cases. A high correlation between the degree of microtia and the frequency of external and middle ear dysplasias was found, in accordance with larger studies of the literature. Inner ear malformations were found less frequently, but without apparent correlation with the degree of microtia. The fact that children with microtia may also have severe inner ear malformations is emphasized.

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