Fois Alessandro G, Arcadu Antonella, Santoru Luigi, Trisolini Rocco, Marras Vincenzo, Ginesu Giorgio C, Canu Sara, Cordero Lorenzo, Diana Gabriella, Pirina Pietro
Department of Respiratory Disease, University of Sassari, viale san Pietro 46/b, 07100 Sassari, Italy.
Thoracic Endoscopy and Pulmonology Unit, Maggiore Hospital, Bologna, Italy.
Multidiscip Respir Med. 2016 Apr 19;11:16. doi: 10.1186/s40248-016-0050-7. eCollection 2016.
Tracheobronchopathia Osteochondroplastica (TBPO) is an uncommon and benign condition characterized by osseous or metaplastic cartilaginous 1-3 mm nodules in the submucosa of the tracheo-bronchial tree. Posterior membranous wall of trachea is typically spared. Ecchondrosis and exostosis nodules can cause chronic inflammation and mucosal metaplasia, stiffness and airway obstruction. The prevalence of this disease, often asymptomatic or associated with nonspecific symptoms, is underestimated, and the mean age at diagnosis is 50 years.
We report a case of a 49 year old male, non-smoker. He was a smith, homeless, born in Romania and reported a diagnosis of asthma since childhood. He was admitted to our Respiratory Unit presenting low-grade fever with profuse sweating, cough, purulent sputum, and ground-glass opacity with irregularity in main bronchi detected by High-Resolution Computed Tomography (HRCT) scan. Fibrobronchoscopy revealed the presence of mucosal irregularities up to the segmental bronchi entrance. Histological examination showed nodules of osseouscartilaginous nature, consistent with TBPO. Microbiological tests of Bronchoalveolar Lavage fluid also revealed an infection by Pseudomonas Aeruginosa.
TBPO is a rare disease characterized by wheezing, cough, hemoptysis, and recurrent pulmonary infections, with typical onset during adulthood. In the case reported, the symptoms began in childhood, although they had been misinterpreted as asthma. Even if childhood-onset is not reported in literature, it is likely that small changes occur in the first few years of life and become more evident in adulthood. The involvement of segmental and sub-segmental bronchi, usually spared in TBPO, could explain the presence of wheezing and non-productive cough reported by our patient since childhood.
气管支气管骨软骨化生（TBPO）是一种罕见的良性疾病，其特征是气管支气管树黏膜下层出现1 - 3毫米的骨性或化生软骨结节。气管后壁膜部通常不受累。软骨瘤和外生骨疣结节可引起慢性炎症、黏膜化生、僵硬和气道阻塞。这种疾病通常无症状或伴有非特异性症状，其患病率被低估，诊断时的平均年龄为50岁。
我们报告一例49岁男性，非吸烟者。他是一名铁匠，无家可归，出生于罗马尼亚，自幼被诊断为哮喘。他因低热、多汗、咳嗽、脓性痰入院，高分辨率计算机断层扫描（HRCT）显示主支气管有磨玻璃样混浊及不规则影。纤维支气管镜检查显示直至段支气管入口处均有黏膜不规则。组织学检查显示为骨软骨性质的结节，符合TBPO。支气管肺泡灌洗液体的微生物学检测还显示有铜绿假单胞菌感染。
TBPO是一种罕见疾病，其特征为喘息、咳嗽、咯血和反复肺部感染，典型发病于成年期。在本报告的病例中，症状始于儿童期，尽管当时被误诊为哮喘。即使文献中未报道儿童期发病情况，但很可能在生命的最初几年就发生了微小变化，并在成年期变得更加明显。段支气管和亚段支气管受累，而在TBPO中通常不累及，这可以解释我们的患者自幼出现喘息和干咳的原因。
