骨化性气管支气管病二例报道并文献复习

Tracheobronchopathia Osteochondroplastica:Two Cases Report and Literature Review

目的探讨骨化性气管支气管病(TO)的临床特点、诊断及治疗。方法报告2例TO患者的临床资料,结合文献分析。结果TO在成年人多发,男性发病率略高,临床症状无特异性。纤维支气管镜是诊断TO的金标准,镜下可见大气道前壁和侧壁多发增生性结节突向管腔,后壁(膜部)通常不受累。胸部CT的敏感性和特异性均很高,在TO的诊断中起重要作用。组织病理活检可见支气管黏膜下软骨和骨组织的存在。目前尚无针对TO的特异性治疗方法,仅少数导致气道重度狭窄的患者需接受手术治疗。结论TO是一种良性疾病,预后较好,肺部CT、纤维支气管镜有特征性改变,可以确诊。

Objective To investigate the manifestations, diagnosis and treatment of tracheobronchopathia osteochondroplastica （TO）. Methods Two cases of TO were described and 76 cases in the medical literature after 2000 were reviewed. Results TO usually manifests in adults, and affects both genders. The clinical presentation of TO is nonspecific. Bronchoscopy remains the gold standard for diagnosing this condition. Hard sessile nodules arising from the anterior and lateral walls of the airway, typically sparing the posterior membrane, are classic appearance that can be easily recognized. The CT scan is more sensitive and specific, which plays an important role in the diagnosis of TO. Bronchial biopsies disclose the abnormal presence of cartilage and bone tissue in the bronchial submucosa. To date there is no specific treatment for the disease. Only a minority of cases develop into significant upper airway obstruction and require invasive procedures to remove or bypass the obstacle on affected airways. Conclusions TO is a stable or slowly progressive benign disease. Chest computed tomography and fiberoptic bronchoscopy are the best diagnostic procedures to identify TO.