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Case report

Tracheobronchopathia osteochondroplastica: four cases

Dursun, Tatar; Gunes, Senol; Atike, Demir; Gulru, Polat

Editor(s): PAN, Cheng; GUO, Li-shao

Author Information
doi: 10.3760/cma.j.issn.0366-6999.2012.16.026
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Abstract

Tracheobronchopathia osteochondroplastica (TO) is characterized by variable numbers of sessile, irregular, bony or cartilaginous submucosal nodules of the tracheal submucosa covered by normal epithelium. Nodules sometimes reach to the main bronchus and rarely to larynx. Nodules are originated from airway cartilage tissue. Posterior membranous wall of trachea is typically protected. The disease is usually asymptomatic and has benign course. It was described first time by Wilks in 1857 in the autopsy of a 38-year-old man who expired of tuberculosis. Though its etiology remains unknown; chronic inflammatory processes or degeneration, metabolic and neoplastic disorders are blamed in the etiology.1 The diagnosis of TO was generally established during the autopsy in the past, but, at present day, it can be recognized in antemortem period by the developed diagnostic possibilities like computed tomography and bronchoscopy.2 We presented the cases that TO should be considered in differential diagnosis of calsific lesions of tracheabronchial system or cases with hemoptysis.

CASE REPORTS

Case 1

Patient was 44 years old, male. He described approximately 300-400 ml bleeding after a sudden cough attack with no prior history. He declared smoking habit for 30 years per packet daily. White blood cell count was found moderate high (white blood cells: 11.3×109/L). Other laboratory tests and physical examination were usual. His pulmonary function tests (arterial blood gases and lung function test) were in normal range. Postero-anterior (PA) chest radiogram pointed out enlargement of hilary lymph nodes and increasing in bronchovascular shadows, two calcific lesions between anterior arms of fourth and fifth ribs in the lower zone. Bilateral hilary, subcarinal, paratracheal calsific lymph nodes and fibrotic changes were detected in each apex; mostly calsific granulamatous nodular lesions about one cm diameter as biggest in lingula, right lower superior lobe and basal segments were seen by thoracic computed tomography (CT). The fiberoptic bronchoscopy (FOB) was performed for investigating the cause of hemoptysis. FOB was revealed the lesions, which were protuberated from the mucosa, giving white reflection, conglomerulated, looking like stiff pearl drops usually smaller than one cm diameter. Half of the lesions were seen in distal trachea, in lateral wall of the right main bronchus and in left main bronchus beginning from carina (Figure 1). Hemorrhagic residues were detected. Because of the stiffness of the tissue, forceps biopsy was hardly performed. TO was diagnosed by histopathological examination of the specimen taken from submucosal metaplastic cartilage and bone tissue (Figure 2). Hemoptysis has lasted in a few days after the hospitalization. Lesions in chest radiogram and CT were evaluated as sequel and chronic changes. Case has followed up and hemoptysis did not recur. Any special treatment was not performed.

Figure 1.
Figure 1.:
FOB views of the case 1.Figure 2. Histological appearance of bronchoscopic biopsy of the first case. Bronchial epithelium, mucosa and submucosa, covered with nodules of mature bone, fat and connective tissue areas (HE staining, Original magnification ×200).Figure 3. Multiple polypoid, small, white lesions covered with front and side walls of trachea, showed by FOB of case 3.Figure 4. Histological appearance of bronchoscopic biopsy of the third case. Lamellar bone ossification in bronchial mucosa (HE staining, Original magnification ×100).Figure 5. CT image of indicating nodulations from tracheal wall into the lumen of case 4.Figure 6. Swollen mucosa with white pearllike lesions along walls except the back wall of the trachea and partly to the main bronchi, beginning just after cords of vocal.

Case 2

Patient was 49 years old, male. He applied to hospital due to fever lasting for 15 days, cough and sputum expectoration. He had been smoking 2-3 cigarettes daily for 10 years and he had a history of nephrectomy due to nephrocalsinosis in 1997. In his physical examination, rales were auscultated in the lower part of his right hemithorax. Erythrocyte sedimentation rate was found 60 mm/h, CRP was calculated 2.46 mg/dl. Other laboratory tests were found in normal range. Pulmonary function test was evaluated normal. PA chest radiogram revealed enlarged right hilus, heterogenic density in the right paracardiac area. Radiological preliminary diagnosis was established as pneumoconiosis or sarcoidosis. FOB was performed for observing lower airways and sampling respiratory secretions and tissue for explaining of unrespondness to antibiotherapy with amoxicilinclavulonic acid lasting two weeks. Reflecting white color, widespread mucosal protuberances looking like pearl drops in the 2/3 of distal lateral and anterior walls of trachea, partly conglomerulated and swollen mucosal lesions in the right main bronchus reaching to the upper lobe bronchus and similar lesions in the left main bronchus were detected. Histopathological examination of biopsy sample was reported as TO. Antibiotherapy was changed to cefuroxime axetil and clarithromycin due to pulmonary infiltration. Clinical symptoms have relieved and radiological lesions were regressed. It is concluded that TO was diagnosed coincidentally while investigating for prolonged lower respiratory infection in that case.

Case 3

Patient was 56 years old, male. Thymoma and myasthenia gravis were diagnosed recently due to complaints of limitation of bilateral external sight, common muscle weakness and shortage of breath upon effort. In his chest X-ray, there was a slight reticulonoduler density in the inferior zones. In his CT of the thorax, a smooth tissue mass about three cm diameter at the upper anterior mediastinal area, loss of ventilation in inferior lobes and consolidation zones including air-bronchograms have been observed. Laboratory tests were in normal range. The arterial blood gas analysis were indicated hypoxia (pH: 7.39, PO2: 64 mm Hg, PCO2: 48 mm Hg, HCO3: 26.6 meq/L, O2 sat: 92.1%) and restrictive findings were established by spirometric test (VC: 1.77 L (44%), FVC: 1.77 L (45%), FEV1: 1.40 (45%), FEV1/FVC: 79%). FOB was performed. Anterior and lateral walls of trachea until the carina were observed to be covered with little, white colored, multiple polipoid lesions (Figure 3). Histopathological examination of biopsy material was assessed as TO (Figure 4). Respiratory insufficiency and pulmonary infection were considered as complication of myasthenia gravis. The case underwent surgery for tymoma and was referred to neurology clinic for treatment of myasthenia gravis. Histopathological examination was reported as TO. The case was considered tymoma associated with TO.

Case 4

Patient was 54 years old, female. She has suffered cough for six months. Physical examination and chest X-Ray were detected in normal limits. Any abnormal laboratory test results were not reported. Otolaringeal examination indicated normal structure. Thoracic CT was revealed protrusions originated from tracheal wall to lumen (Figure 5). FOB was performed to investigate the cause of chronic cough. White, rough, raised from mucosa, pearl like drops lesions beginning just after cord vocals and along tracheal walls except back wall, partially main bronchi were detected (Figure 6). Biopsy samples were taken from lesions and were interpreted as TO. TO was considered the cause of chronic cough.

DISCUSSION

TO was reported 0.01%-4.2% in the cases who underwent bronchoscopic examination (Table 1).3–9 Our patient (three male and one female) were in their fifth and sixth decades. In literature, TO was encountered in males three times higher than that in females. It was generally diagnosed over 50 years old.10 However, young adults and children can also be affected.1 Any descriptive characteristics of anamnesis were not defined in studies.

Table 1
Table 1:
Incidence of TO in different publications

Complaints of patients were variable according to accompanied disorder. Major complaints are chronic cough and hemoptysis.4,11 Sometimes nodules make polipoid masses causing dyspnea and wheezing.8 The chronic cough was found 54% in a study which investigated 41 cases with TO.12 In another study, cough was the most common complaint in 66% of cases and hemoptysis is in second order (60%).16 Reason of the hemoptysis is generally found secondary to the ulceration or infection of nodules.1,2 Recurrent lower respiratory infections, dryness of throat and dysphonia are less common complaints in TO.1,4,7 TO can make patient sensitive for opportunistic infections like atypical mycobacterium infections and botromycosis due to impairing local airway defense mechanisms (failure of mucociliary activity).1 TO also should be considered as a possible pathology in the cases that have history of intubation problems.11 Chronic cough and hemoptysis were specific complaints at two our cases as indicated in literature. TO were diagnosed by coincidence in other cases while investigating primary disease.

Amiliodosis, endobronchial sarcoidosis, calsific tuberculosis lesions, papillamatosis, tracheabronchial calsinosis, bronchial and tracheal tumors, COPD and asthma bronchiole should be considered in differential diagnosis.9,11 Tumor of thyroid, tymoma, atrophic rhinitis, lymphoma and epidermal cysts have been accompanying diseases in some cases.13 One of our cases was stricken of myasthenia gravis. There is another reported case with myasthenia gravis with TO in the literature.14

Strict diagnostic test seems to be FOB. In half of our cases FOB was performed primary problems that could be dealing with TO reasons. Almost same macroscopic lesions were revealed by FOB in our cases. But dissemination of lesions was found different. Lesions were existed anterior and lateral walls of trachea. Posterior wall was intact. This finding was remarkable specification for TO in agreement with most reported cases.5,6 CT scan sometimes indicates the calcific lesions, constriction and irregularity of airways. Although providing biopsy specimen from the nodules is not necessary for diagnosis, when performed, heterotypic bone formation is seen histopathologically in 60% of cases.1,6

Lesions do not carry any malign transformation potential. They progress slowly and rarely need treatment. In long time period, obstruction and recurrent infections might be occurred.1,9 Certain treatment approach was not described for TO, palliative treatments are given.4 Cases were advised to follow up by spirometric tests to evaluate progressing restriction pattern. Antibiotic therapy for infectious complications, surgical resection, forceps biopsies, stent applications and laser therapy for obstructive lesions were mentioned in literature.3,14 In other case, it was reported sub-glottic stenosis caused by TO which required an urgent tracheostomy and afterwards laser debulking of stenosis.15

In conclusion, we report the cases because of TO is an unusual disease and the diagnosis is seldom made because of the chronic and asymptomatic nature of the condition. TO should be considered in case of unsolved cough and hemoptysis disorders.

REFERENCES

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Keywords:

airway obstruction; chronic cough; fiberoptic bronchoscopy; hemoptysis; respiratory metaplasia; tracheobronchopathia osteochondroplastica

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