巨气管支气管症三例并文献复习

Tracheobronchomegaly： a report of 3 cases and literature review

目的分析3例巨气管支气管症（tracheobronchomegaly，TBM），即莫一昆综合征（Mounier—Kuhnsyndrome）患者的临床特征，以提高对该病的认识，从而有利于早期诊断和治疗并发症，改善预后。方法回顾性分析北京朝阳医院确诊的3例TBM患者的临床特点、影像学和病理特征，并结合文献报道进行总结。结果3例患者均为男性，年龄分别为58、65和71岁。从出现症状到确诊的时间，分别为2个月、1年和2年。主要症状为反复咳嗽和咳痰，偶有咯血；疾病后期肺功能受损时出现呼吸困难和呼吸衰竭的相应表现。所有患者均通过x线胸片和胸部CT发现显著的气管、支气管扩张确诊。经抗感染治疗病情均能控制。TBM是一种罕见的、先天性疾病，特征性的改变为气管、主支气管的弹力纤维和平滑肌组织萎缩、纤细，导致气管支气管显著扩张，可合并远端支气管扩张。男性好发，发病年龄多为30～40岁。临床表现无特异性，常见症状为反复咳嗽和咳痰，偶有咯血。影像学特征为气管和主支气管扩张。当疾病进一步进展，可出现呼吸困难或呼吸衰竭。治疗主要是预防和控制感染，原发病缺乏有效的治疗手段。结论早期诊断并及时控制反复发生的支气管肺部感染，可以阻止TBM患者肺功能的下降，对改善预后至关重要。

Objective To analyze the clinical, radiological, and pathological characteristics of tracheobronchomegaly （TBM, Mounier-Kuhn syndrome ）. Methods The clinical, radiologieal and pathological characteristics of 3 cases of TBM were analyzed, and the literatures were reviewed. Results All 3 patients were men, between the age of 58 -71 years old. From the onset to diagnosis, the shortest time was 2 months, and the longest 43 years. The most usual presentations included recurrent cough and sputum, and occasional haemoptysis. In the advanced stage of the disease, patients would present shortness of breath and the symptoms associated with respiratory failure because of the reduction in pulmonary function. All the diagnoses were confirmed by X-ray and CT of the chest finding that the trachea and the main bronchi dilated markedly. After anti-infection treatment, all patients recovered. Mounier-Kuhn syndrome was a rare congenital abnormality characterized by atrophy or absence of elastic fibers and thinning of smooth muscle layer in the trachea and main bronchi. These airways were thus flaccid and markedly dilated on inspiration and collapsed on expiration. The usual presentation was recurrent respiratory tract infections with a broad spectrum of functional impairment ranging from minimal disease with preservation of lung function to severe disease in the form of bronchiectasis, emphysema and pulmonary fibrosis, ultimately culminating in respiratory failure and death. Computed tomography scan of the chest was used for the diagnosis. Treatment was mainly supportive with chest physiotherapy and antibiotics. Conclusions Mounier-Kunh syndrome should be suspected in patient with recurrent respiratory infections and chronic sputum production. A careful analysis of the central airways at the chest radiograph of these oatients is recmired.