Celik Burcin, Bilgin Salih, Yuksel Canan
Department of Thoracic Surgery, 19 Mayis University Medical School, 55139 Samsun, Turkey.
Tex Heart Inst J. 2011;38(2):194-6.
Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Diagnosis is typically accomplished with the use of computed tomography and bronchoscopy, as well as pulmonary function testing. Patients may be asymptomatic; however, symptoms can range from minimal with preserved lung function to severe respiratory failure. Therapy, if any, is supportive but minimal. Surgery rarely has a place in the treatment of Mounier-Kuhn syndrome.Herein, we report the case of a 58-year-old man with chronic obstructive pulmonary disease who had a chronic cough, increased sputum production, and chest pain. Thoracic computed tomography showed tracheal dilation (diameter, 34 mm) and multiple diverticula in the posterior region of the trachea. Fiberoptic bronchoscopy revealed enlarged main bronchi, the dilated trachea, and prominent tracheal diverticula. Pulmonary function testing disclosed impaired respiratory function. Histopathologic examination of biopsy specimens from the bronchi and the tracheal wall supported the diagnosis of Mounier-Kuhn syndrome. The patient was released from the hospital and his condition was monitored for 2 years, during which time he developed no lower respiratory tract infections.Regardless of radiologic findings that suggest recurrent lower respiratory tract infection, we recommend that Mounier-Kuhn syndrome be considered in the differential diagnosis.
穆尼耶 - 库恩综合征,即气管支气管巨大症,是一种罕见的临床和影像学病症,其特征为显著的气管支气管扩张和反复的下呼吸道感染。诊断通常通过计算机断层扫描、支气管镜检查以及肺功能测试来完成。患者可能无症状;然而,症状范围可从肺功能保留时的轻微症状到严重呼吸衰竭。治疗(若有)以支持治疗为主,但力度较小。手术在穆尼耶 - 库恩综合征的治疗中很少有用。在此,我们报告一例58岁患有慢性阻塞性肺疾病的男性病例,该患者有慢性咳嗽、痰液增多和胸痛症状。胸部计算机断层扫描显示气管扩张(直径34毫米)以及气管后部有多个憩室。纤维支气管镜检查显示主支气管增粗、气管扩张以及明显的气管憩室。肺功能测试显示呼吸功能受损。对支气管和气管壁活检标本的组织病理学检查支持穆尼耶 - 库恩综合征的诊断。该患者出院后病情监测了2年,在此期间他未发生下呼吸道感染。尽管影像学检查结果提示反复下呼吸道感染,但我们建议在鉴别诊断中考虑穆尼耶 - 库恩综合征。