Alternative titles; symbols
SNOMEDCT: 37615000, 54675009; ORPHA: 3348;
Prakash et al. (1989) described a mother and daughter with tracheopathia osteoplastica, also known as tracheobronchopathia osteochondroplastica. This disorder is characterized by cartilaginous or bony projections into the tracheobronchial lumen, with sparing of the posterior membranous portion of the tracheobronchial tree. Symptoms may, however, include dyspnea, coughing, hemoptysis, hoarseness, and wheezing. Tomography of the trachea may show beaded calcification of the tracheobronchial cartilages. Bronchoscopy is diagnostic. Histologically, the abnormal growths show heterotopic bone formation. The mother recorded by Prakash et al. (1989) had recurrent episodes of pneumonia attributable to bronchial obstruction by bony projections. In the daughter, removal of large lesions that obstructed the upper part of the trachea relieved dyspnea. No other reports of familial occurrence were found. Prakash et al. (1989) stated that this abnormality is detected in about 1 in 2,000 bronchoscopies.
Prakash, U. B. S., McCullough, A. E., Edell, E. S., Nienhuis, D. M. Tracheopathia osteoplastica: familial occurrence. Mayo Clin. Proc. 64: 1091-1096, 1989. [PubMed: 2509829] [Full Text: https://doi.org/10.1016/s0025-6196(12)64978-7]