Tracheobronchopathia osteochondroplastica (TO) is a rare benign disease of unknown cause, in which multiple

cartilaginous Cartilage is a resilient and smooth type of connective tissue. Semi-transparent and non-porous, it is usually covered by a tough and fibrous membrane called perichondrium. In tetrapods, it covers and protects the ends of long bones at the joints ...

or bony submucosal

nodule Nodule may refer to: * Nodule (geology), a small rock or mineral cluster * Manganese nodule, a metallic concretion found on the seafloor *Nodule (medicine), a small aggregation of cells *Root nodule Root nodules are found on the roots of plants, ...

s project into the

trachea The trachea (: tracheae or tracheas), also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all animals' lungs. The trachea extends from ...

and proximal

bronchi A bronchus ( ; : bronchi, ) is a passage or airway in the lower respiratory tract that conducts air into the lungs. The first or primary bronchi to branch from the trachea at the carina are the right main bronchus and the left main bronchus. Thes ...

. The nodules usually spare the posterior wall of the airway because they are of cartilaginous origin, while the posterior wall of the airway is membranous (does not contain cartilage). This is as opposed to tracheobronchial

amyloidosis Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. There are several non-specific and vague signs and symptoms associated with amyloidosis. These include fatigue, peripheral edema, weigh ...

, which does not spare the posterior wall. It usually occurs in men around their fifth decade of life, as opposed to tracheobronchial papillomatosis due to HPV infection, which usually occurs in younger patients. TO can cause airway obstruction, bleeding and chronic cough. Treatment involves the use of

bronchodilator A bronchodilator or broncholytic (although the latter occasionally includes secretory inhibition as well) is a substance that dilates the bronchi and bronchioles, decreasing resistance in the respiratory airway and increasing airflow to the lun ...

s, and physical dilatation by

bronchoscopy Bronchoscopy is an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument (bronchoscope) is inserted into the airways, usually through the nose or mouth, or occasionally through a tr ...

. The patients are also more prone to post-obstructive

pneumonia Pneumonia is an Inflammation, inflammatory condition of the lung primarily affecting the small air sacs known as Pulmonary alveolus, alveoli. Symptoms typically include some combination of Cough#Classification, productive or dry cough, ches ...

and chronic lung infection in severe cases.

Diagnosis

Differential diagnosis

The differential of TO includes amyloidosis, which is typically circumferential, papillomatosis, though this usually occurs in younger patients and can cause lung cavitation when disseminated,

granulomatosis with polyangiitis Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), after Nazi German physician Friedrich Wegener, is a rare, long-term, systemic disorder that involves the formation of granulomas and vasculitis, inflammatio ...

, though this is circumferential as well and often involves distal lung cavitation as well.

Relapsing polychondritis Relapsing polychondritis is a systemic disease characterized by repeated episodes of inflammation and in some cases deterioration of cartilage. The disease can be life-threatening if the respiratory tract, heart valves, or blood vessels are affect ...

can also spare the posterior wall, though it is not typically nodular in appearance.

Treatment

References

External links

{{Medical resources , ICD10 = J98.0 , ICD9 = , ICDO = , OMIM = 189961 , DiseasesDB = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , MeSH = , GeneReviewsNBK = , GeneReviewsName = , Orphanet = Trachea Respiratory diseases