Mathlouthi A, Ben Rehouma C, Ben M'Rad S, Dumon J F, Labbene N, Driss B, Sellami M, Djenayah F
Centre Laser, Service du Dr J. F. Dumon, Hôpital Sainte-Marguerite, Marseille, France.
Rev Pneumol Clin. 1993;49(3):156-62.
Tracheobronchobathia osteoplastica (TBO) is a benign disease first described in 1855 and rather rare since only 371 cases have been reported so far. The disease is characterized by the presence of subepithelial osteocartilaginous focal lesions without any relation to tracheal rings. The mechanism of its occurrence remains controverted. We report a case of TBO which is particular in that is was associated with atrophic rhinitis, polydactylia and disorder of oesophageal motricity. From a compilation of 113 cases published by French and Anglo-Saxon authors since 1970, it appears that the clinico-radiological and laboratory picture is of little help in the positive diagnosis which in fact rests on three key-points: bronchial endoscopy, pathological examination of bronchial biopsy fragments and thoracic imaging techniques including computerized topography and magnetic resonance imaging. The usually benign course of the disease can be marked by severe complications, such as infections, haemorrhages, acute dyspnoea by extension to the larynx or significant narrowing of the tracheal lumen which may need tracheotomy or surgical resection of the lesions.
骨化性气管支气管病（TBO）是一种良性疾病，于1855年首次被描述，目前较为罕见，因为迄今为止仅报道了371例。该疾病的特征是存在上皮下骨软骨局灶性病变，与气管环无关。其发病机制仍存在争议。我们报告了一例特殊的骨化性气管支气管病病例，该病例伴有萎缩性鼻炎、多指畸形和食管动力障碍。从1970年以来法国和盎格鲁 - 撒克逊作者发表的113例病例汇编来看，临床放射学和实验室检查结果对明确诊断帮助不大，实际上确诊依赖三个关键点：支气管内镜检查、支气管活检碎片的病理检查以及胸部成像技术，包括计算机断层扫描和磁共振成像。该疾病通常呈良性病程，但可能出现严重并发症，如感染、出血、因病变蔓延至喉部导致的急性呼吸困难或气管腔显著狭窄，这种情况下可能需要气管切开术或手术切除病变。
