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Airways Disease

Congenital Tracheal Anomalies


Tracheoceles
Paratracheal air cysts
True diverticula
Herniation of the tracheal air column through a weakened posterior
tracheal membrane
Occur almost exclusively in the cervical trachea
Usually asymptomatic
CT: circular lucencies along the right posterolateral trachea at the
thoracic inlet
Tracheal bronchus
Bronchus suis
Consists of an accessory bronchus to all or a portion of the right
upper lobe that arises from the right lateral tracheal wall within 2 cm
of the tracheal carina
Most often supplies the apical segment of the right upper lobe
Asymptomatic
Usually an incidental finding on chest CT
Association with congenital tracheal stenosis and an aberrant left PA
Focal Tracheal Disease
Extrinsic Mass Effect
Most common cause: tortuous or dilated aortic arch or
brachiocephalic artery
Because the tracheal cartilage provides resiliency, extrinsic masses
tend to displace the trachea without narrowing its lumen
Traction deformity of the trachea is generally seen in cicatrizing
processes that asymmetrically affect the lung apices (i.e., postprimary
tuberculosis (TB), histoplasmosis, and radiation fibrosis)
Focal Tracheal Stenosis
May result from inflammatory disorders that affect the tracheal or
central bronchial walls
From cartilaginous damage or the development of granulation tissue
and fibrosis from a tracheostomy or at the site of a previously inflated
endotracheal tube balloon cuff
Typical hourglass deformity on frontal radiographs
Extrathoracic tracheomalacia (from the site of a prior tracheostomy)
demonstrate tracheal narrowing on inspiration
Intrathoracic tracheomalacia (prior endotracheal intubation) have
tracheal narrowing on expiration
Wegeners granulomatosis
Tracheal narrowing + upper airway and renal involvement + biopsy
Tx: Cyclophosphamide therapy

Endotracheal and endobronchial TB


Usually associated with cavitary TB
Production of large volumes of infected sputum predisposes to tracheal and
central bronchial infection
Tracheal scleroma
infection with Klebsiella rhinoscleromatis
Chronic phase, intense granulation tissue and fibrosis lead to stenosis of the
nasal cavity, pharynx, larynx, and upper trachea
Upper trachea shows irregular nodular narrowing, which may extend to
involve the length of the trachea
Biopsy: granulation tissue containing large foamy histiocytes filled with the
causative organism (Mikulicz cells)
Tx: Antibiotic therapy
Focal tracheal dilatation
Caused by congenital or acquired abnormalities of the elastic
membrane or cartilaginous rings of the trachea.
Localized tracheal dilatation may be seen with tracheoceles, with
acquired tracheomalacia related to prolonged endotracheal
intubation, or as a result of tracheal traction from severe unilateral
upper lobe parenchymal scarring
Diffuse Tracheal Disease
Diffuse Tracheal Narrowing
Congenital tracheal stenosis
Rare
incomplete septation of the cartilage rings,
Napkin ring trachea = long segment tracheal narrowing
Often associated with other congenital cardiovascular anomalies
Anomalous origin of the left PA from the right PA (PA sling)
Anomalous origin of the right upper lobe bronchus from the trachea (tracheal
bronchus or bronchus suis).
Saber-sheath trachea
Fixed deformity of the intrathoracic trachea
Coronal diameter is diminished to less than two-thirds of the sagittal
diameter.
Tracheal wall is uniformly thickened
Calcification of the cartilaginous rings is present in most cases.
Exclusively affects older men with functional evidence of chronic obstructive
pulmonary disease.
Amyloidosis
Deposition of a fibrillar proteinpolysaccharide complex in various organs.
Mass-like circumferential deposits that irregularly narrow the tracheal lumen
are best demonstrated on CT and can result in recurrent atelectasis and
pneumonia.
Diagnosis is made by the presence of typical proteinpolysaccharide deposits
demonstrated following Congo red staining of tracheal or bronchial wall
biopsy specimens.
Apple-green birefringence when viewed under polarized light
Tracheobronchopathia osteochondroplastica
Rare
Characterized by the presence of multiple submucosal osseous and
cartilaginous deposits within the trachea and central bronchi of elderly men
Diagnosis is generally made on bronchoscopy and CT
Calcified plaques can be seen involving the anterior and lateral walls of the
trachea.
Sparing of the membranous posterior wall of the trachea, which lacks
cartilage, is a helpful feature that distinguishes this entity from
tracheobronchial amyloid
Usually asymptomatic
May have recurrent infection related to bronchial obstruction by the masses.
Relapsing polychondritis
Systemic autoimmune disorder that commonly affects the cartilage of the
earlobes, nose, larynx, tracheobronchial tree, joints, and large elastic arteries.
Respiratory complications secondary to involvement of the upper airway
cartilage accounts for nearly50% of all deaths from this condition.
Diagnosis: recurrent inflammation at two or more cartilaginous sites,
Most commonly the pinnae of the ear (producing cauliflower ears) and the bridge of the
nose (producing a saddlenose deformity).
Radiographs and CT: diffuse smooth thickening of the wall of the trachea and
central bronchi with narrowing of the lumen
Diffuse Tracheal Dilatation
Tracheobronchomegaly (MounierKuhn syndrome)
Congenital disorder of the elastic and smooth muscle components of the tracheal
wall
Association with EhlersDanlos syndrome and cutis laxa
Found almost exclusively in men under the age of 50.
Abnormal compliance of the trachea and central bronchi leads to central bronchial
collapse during coughing.
Symptoms are indistinguishable from those associated with chronic bronchitis and
bronchiectasis.
On frontal radiographs, the trachea and central bronchi measure greater than 3.0 cm
and 2.5 cm, respectively, in coronal diameter.
The trachea has a corrugated appearance caused by the herniation of tracheal
mucosa and submucosa between the tracheal cartilages
Tracheobronchomalacia (TBM)
Congenital or acquired defect of tracheal cartilage
Congenital disorders
Relapsing polychondritis
EhlersDanlos syndrome
Mucopolysaccharidosis
Acquired TBM
More common
Most often the result of prolonged intubation, prior tracheostomy, and extrinsic
tracheal compression by mediastinal masses and vascular anomalies
Imaging: excessive airway collapse on expiration, seen best on CT performed at total
lung capacity (i.e., inspiration)
A reduction in the cross-sectional area of the trachea exceeding 50% on the
expiratory CT, particularly if there is a crescentic frown-like configuration to the
trachea in cross section, is strongly suggestive
Tracheal and Bronchial Injury
Most often seen with blunt chest trauma from a deceleration-type
injury
Concomitant aortic laceration, great vessel injury, or rib (particularly
an upper anterior rib), sternum, scapula, or vertebral fracture is the
rule
Mechanism: forceful compression of the central tracheobronchial
tree against the thoracic spine during impact
Proximal main bronchi (80%) or distal trachea (15%) within 2 cm of
the tracheal carina; the peripheral bronchi are involved in 5% of
cases.
Most common form: Horizontal laceration or transection parallel to
the tracheobronchial cartilage
Early chest radiographs: presence of pneumothorax and
pneumomediastinum, particularly in a patient not receiving
mechanical ventilation
Pneumothorax fails to respond to chest tube drainage owing to a
large air leak at the site of airway interruption
Subtended lung remains collapsed against the lateral chest wall
(fallen lung sign)
Aberrant endotracheal tube or an overdistended balloon cuff is a
further clue
Definitive diagnosis is by bronchoscopy
MDCT with three-dimensional reconstruction with shaded surface
display may be useful in patients who develop bronchial occlusion or
stenosis because of a delay in diagnosis

Penetrating tracheal injuries usually involve the cervical trachea


Result from gunshot or stab wounds to the neck.
Injury to the intrathoracic trachea is usually associated with fatal
penetrating cardiovascular injury
Broncholithiasis