急速に気管狭窄をきたしたＴｒａｃｈｅｏｂｒｏｎｃｈｏｐａｔｈｉａ Ｏｓｔｅｏｃｈｏｎｄｒｏｐｌａｓｔｉｃａの１症例

Abstract

Tracheobronchopathia osteochondroplastica is a rare disease first described by Wilks in 1857. In Japan, it was first described by Kidokoro in 1938. To date, about 370 cases of this disease have been reported in the world literature, while in Japan 140 cases have been reported. In the past, such cases were often diagnosed at autopsy, but an increasing number of patients with this disease are being diagnosed before death by bronchoscopy. The following is a report on a case of tracheobronchopathia osteochondroplastica.A 42-year-old man had symptoms of dyspnea and loss of consciousness. Artificial respiration using a tracheal tube was instituted to improve consciousness. A tracheotomy was then deemed necessary. When the tracheotomy and cannulation was performed, the tracheal wall was found to be hard and ossified between the third tracheal cartilage and the subglottic space. White ossiferous nodular lesions were observed. Histopathological findings from a specimen of the nodular lesions showed submucosal bony and cartilaginous tissue covered with tracheal epithelium. CT findings revealed calcified nodules involving the anterior and lateral aspects of the tracheal and bronchial walls. Therefore, tracheobronchopathia osteochondroplastica was diagnosed.To improve the tracheal stenosis between the tracheal stoma and the subglottis, we resected the nodular lesions with a Nd-YAG laser. Residual nodular lesions did not enlarge after this treatment, but the patient died from DIC.Although this disease was been reported to be a very slowly progressing disease, our case showed a distinct progress within a short period. It was suggested that the rapid growth of the nodular lesions in this case was caused by physical stimulation. Therefore, we considered surgical and Nd-YAG laser treatments to be advisable for the excision of the nodular lesions in tracheobronchopathia osteochondroplastica.