Tracheopathia osteoplastica (TPO) is a disease of the trachea that is not well recognized and under diagnosed. We present a case of TPO with the novel treatment of tracheal stent placement under flexible bronchoscopy.
A 65-year-old Caucasian male presented with persistent cough and progressive dyspnea over 10 months. He reported decreased exercise tolerance but denied orthopnea, chest pain, pleuritic pain, weight loss and other constitutional symptoms. He took multiple courses of antibiotics for intermittent fever and purulent sputum. Past medical history included rheumatic fever, asbestos pleural plaques, chronic bronchitis, and moderate copd after a 60 pack-year tobacco history. He had no family history of pulmonary disease. He has no known drug allergies. Medications included prednisone 5mg, guaifenex, albuterol and atrovent nebulizer therapy, and flovent. Physical examination revealed a healthy male. Blood pressure was 137/75, pulse of 87, respiratory rate of 24, and oxygen saturation of 97% on 2L nasal cannula. His lungs had coarse breath sounds with good air movement, no audible stridor, normal inspiratory to expiratory ratio. He had neither clubbing nor peripheral edema. The remainder of his exam was normal. Laboratory data noted normal blood work. Pulmonary function tests demonstrated an FEV1=1.8, FVC=4.0 with ratio of 46%, TLC=8.1 (128%), DLCO=14 (66%) and normal flow-volume loops. Chest CT revealed tracheomalacia. Sputum cultures were negative. However, flexible bronchoscopy revealed marked mid- to distal tracheal stenosis with nodules and cartilaginous tracheal ring hypertrophy, and fishmouthing of the right and left main ostia. Needle biopsies revealed cartilaginous hyperplasia.
Differential diagnosis for tracheal stenosis includes malignancy (bronchogenic, laryngeal, esophageal and thyroid carcinomas, Hodgkin’s lymphoma), endotracheal intubation or tracheostomy removal, idiopathic laryngealtracheal stenosis, tuberculosis tracheobronchial stenosis, mucopolysaccharidoses, relapsing polychondritis, Sjogren’s syndrome, Wegener’s granulomatosis, tracheal amyloidosis, saber sheath trachea, and TPO. Pathologic results of cartilaginous hyperplasia established the diagnosis of TPO. TPO is a segmental, degenerative disorder of the tracheobronchial tree characterized by multiple submucosal cartilaginous and osseous nodules causing narrowing of the upper respiratory tract(1). TPO typically involves the first tracheal ring to the carina, but sparing the posterior wall. There is neither gender nor familial predominance. Etiology of TPO is unknown - theories range from elastic tissue neoplasia to chemical or mechanical irritation inciting cartilaginous and osseous metaplasia(2). As the disorder is chronic and often asymptomatic, estimates of incidence vary between 1/2000 bronchoscopies and 1/400 at autopsy(3). Symptoms include cough, dyspnea, chronic bronchitis, purulent sputum, hemoptysis and airway compromise(4). Abnormalities of pulmonary function tests depend on the degree of airway stenosis and can be misleadingly normal. Therefore, diagnosis is dependent on bronchoscopy and pathology. To date, no specific medical therapy is available, with the mainstay of treatment hydration, humidification, mucolytics, antibiotics and expectorants. Optimal management of airway compromise entails rigid bronchoscopy with dilation and removal of obstructing spurs. Use of laser therapy has shown mixed results(5,6). Complete segmental tracheal resection was reported as treatment in one case of massive TPO(7).
We report the first case of TPO treated successfully by tracheal and bronchial stenting with flexible bronchoscopy. At recent follow-up, our patient’s dyspnea remains resolved.
D.K. Loo, None.