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An Unusual Cause of Spontaneous Pneumomediastinum: The Mounier-Kuhn Syndrome

机译:自发性纵隔纵隔的异常原因:穆尼尔-库恩综合征

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摘要

Mounier-Kuhn syndrome is a rare clinical and radiologic condition. It is characterized by tracheal and bronchial dilation. Diagnosis is made by computed tomography and bronchoscopy. An 81-year-old man presenting with an acute chest pain was referred to the pulmonology department. His chest computed tomographic scan showed a tracheobronchomegaly with an increase in the diameter of both the trachea and right and left main bronchi, associated with pneumomediastinum and fibrosis. Fiberoptic bronchoscopy revealed enlarged trachea and both main bronchi. These findings are consistent with a diagnosis of Mounier-Kuhn syndrome. Besides considering this long-neglected “orphan disease” when diagnosing spontaneous pneumomediastinum, clinicians should also be aware of an underlying Mounier-Kuhn syndrome in patients with recurrent respiratory infections, in order to avoid complications associated with the disease.
机译:Mounier-Kuhn综合征是一种罕见的临床和影像学疾病。它的特征是气管和支气管扩张。通过计算机断层扫描和支气管镜检查进行诊断。一名出现急性胸痛的81岁男子被转诊至肺科。他的胸部计算机断层扫描显示,气管支气管肿大,气管以及左右主支气管直径增大,与纵隔和纤维化有关。纤维支气管镜检查显示气管和主支气管均增大。这些发现与Mounier-Kuhn综合征的诊断是一致的。在诊断自发性纵隔气肿时,除了考虑这种长期以来被忽视的“孤儿病”外,临床医生还应该意识到反复呼吸道感染患者的潜在Mounier-Kuhn综合征,以避免与该病相关的并发症。

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