Biomedical subjects

D M Nienhuis

Publications and source records attributed to D M Nienhuis.

2 recordsLinked to original sources

PubMed1990

Tracheobronchopathia osteochondroplastica.

Tracheobronchopathia osteochondroplastica is an unusual disease of obscure causation characterized by cartilaginous or bony outgrowths into the lumen of the tracheobronchial tree. Our retrospective review of 15 patients, 8 of whom were women, revealed a mean age of 63.5 years. The most common symptoms were cough (66%), hemoptysis (60%), dyspnea on exertion (53%), and wheeze (30%). Thirteen percent of the patients were asymptomatic. Chest radiography was not helpful in the diagnosis. Tracheal tomography revealed typical beaded intraluminal calcification in 4 of the 12 patients tested. Mirror laryngoscopy initially revealed the abnormalities in 30% of the patients, and bronchoscopy confirmed the diagnosis and determined the extent of the disease in all patients. Even though upper airway involvement has been thought to be uncommon, 40% of our patients demonstrated abnormalities of the larynx and upper trachea. Histologic confirmation of heterotopic bone formation was obtained in 60% of the patients. Pulmonary function tests showed mild obstructive lung disease. There were no deaths directly attributable to the disease. Treatments attempted included cryotherapy, laser excision, external beam irradiation, and bronchoscopic removal of the obstructing lesions.

Adult↗

PubMed1989

Tracheopathia osteoplastica: familial occurrence.

Tracheopathia osteoplastica is an unusual disease characterized by cartilaginous or bony projections into the tracheobronchial lumen, with sparing of the posterior membranous portion of the tracheobronchial tree. The cause of this disorder is unknown. The diagnosis is seldom made because of the chronic and asymptomatic nature of the condition. More than 90% of the cases are diagnosed at postmortem examination. Symptoms may include dyspnea, coughing, hemoptysis, hoarseness, and wheezing. Tomography of the trachea may reveal beaded calcification of the tracheobronchial cartilages. Bronchoscopy is diagnostic. Histologically, the abnormal growths show heterotopic bone formation. No known treatment is available. We describe two female patients, one with hemoptysis and another with asthmalike symptoms, in both of whom bronchoscopy established the diagnosis of tracheopathia osteoplastica. The first patient had recurrent episodes of pneumonia attributable to bronchial obstruction by bony projections. In the second patient, removal of large lesions that obstructed the upper part of the trachea relieved the dyspnea. Of interest is the fact that the first patient was the biologic mother of the second. To our knowledge, this constitutes the first report of familial occurrence of tracheopathia osteoplastica.

Aged↗