CASE 18322 Published on 09.10.2023

Breath-taking surprises: Tracheobronchopathia osteochondroplastica unveiled

Section

Chest imaging

Case Type

Clinical Cases

Authors

Inês Martinho 1, João Vieira 2, Ana Catarina Vieira 1, Anabela Braga 1, Alberto Vieira 1

1 Department of Radiology, Hospital CUF Porto, Portugal

2 Department of Radiology, Hospital do Divino Espírito Santo, Ponta Delgada, Portugal

Patient

79 years, male

Categories
Area of Interest Respiratory system, Thorax ; Imaging Technique CT
Clinical History

A 79-year-old male with no significant medical history, except for previous COVID-19 infection, and a history of smoking, presents with chronic cough with sputum and dyspnea that had worsened in the past 6 months. A chest x-ray was normal, and he was referred for further evaluation with Computed Tomography (CT).

Imaging Findings

CT revealed scalloping and irregular thickening of the tracheal wall, with scattered calcified and non-calcified submucosal nodules, arising from the anterolateral walls of the trachea and also extending down to the proximal portion of the left main stem bronchus. Sparing of the posterior wall was noticed and these findings caused only mild distortion and narrowing of the airway lumen.

Discussion

Tracheobronchopathia osteochondroplastica (TO) is a rare and idiopathic benign condition characterized by the development of multiple osseous and cartilaginous sessile submucosal nodules of variable sizes, along the lower two-thirds of trachea and central bronchi, typically sparing the membranous portion of the trachea (posterior wall). These nodules project into the tracheobronchial lumen, causing varying degrees of airway narrowing [1,2].

The aetiology remains unclear, although chronic inflammation of the airways, such as in COPD or smoking, is believed to contribute to its development [3-5].

The prevalence of TO is not well established, as it is often undiagnosed due to similar clinical presentation with other conditions such as asthma or COPD [6]. Three large retrospective studies reported its incidence around 0,11% among patients undergoing bronchoscopy [7-9]. TO is typically diagnosed in the sixth/seventh decade of life [1,10], with no gender preference [3,5,11].

The disease is usually asymptomatic and progresses slowly, often discovered incidentally during intubation or bronchoscopy [12-14].

Clinical manifestations of airway obstruction can develop, such as chronic cough, suppuration, dyspnea, or hemoptysis due to ulceration of the nodules, as well as recurrent respiratory infections, due to impaired mucociliary clearance [4,7,15,16].

The severity of symptoms and pulmonary function tests depend on the degree of obstruction, ranging from a normal spirometry to an obstructive pattern in patients with extensive disease [13,15].

CT is considered the most reliable imaging method to screen tracheal disorders, being very sensitive in showing the submucosal nodules, with or without calcification. It allows the distinction from other conditions with overlapping findings, but which instead present with posterior wall involvement and circumferential thickening such as amyloidosis and granulomatosis with polyangiitis. Conversely, if sparing of the posterior wall is also seen but instead of tracheal submucosal nodules/focal coarse calcification, CT shows smooth tracheal thickening, relapsing polychondritis requires consideration [2,10,17-20]. Besides, CT can demonstrate disease extension and detect complications such as post-obstructive pneumonia/collapse [2,21,22].

Although bronchoscopy is the gold standard for diagnosis, some authors consider that pathognomonic tomodensitometric findings may be sufficient if bronchoscopy is not feasible [23,24].

TO may be more prevalent than initially believed, and due to the resemblance in presentation to common conditions, it is likely to be misdiagnosed. Therefore, it’s important to increase the awareness of this entity among radiologists, who should consider this diagnosis in patients with chronic respiratory symptoms and recurrent pulmonary infections. CT is the main method for tracheal evaluation, and the presence of calcified nodules with sparing of the posterior wall is the key to diagnosis.

Differential Diagnosis List
Tracheobronchopathia osteochondroplastica
Relapsing polychondritis
Tracheobronchial amyloidosis
Tracheolaryngeal papillomatosis
Granulomatosis with polyangiitis
Final Diagnosis
Tracheobronchopathia osteochondroplastica
Case information
URL: https://www.eurorad.org/case/18322
DOI: 10.35100/eurorad/case.18322
ISSN: 1563-4086
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