Tracheobronchomalacia

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TRACHEOBRONCHOMALACIA
Mita Sanghavi Goel, M.D.
October 8, 2002
Description
 First described in 1952 in 3 infants.
 Caused by weakness of the tracheal wall due to softening of cartilage.
 Often a self-limited disease of children that resolves by the age of 3 years.
Pathophysiology
 Dynamic collapse of the airways during expiration.
 Excessive dynamic compression of the major conducting airways and loss of laminar
airflow cause increased airways resistance and effort of breathing, prolonged expiration,
and distal air trapping.
 Because of inefficient cough mechanism, affected patients retain secretions and are at
increased risk of developing recurrent pneumonia, bronchiectasis, and lung scarring.
Clinical Presentation
Intrathoracic lesion
 Chronic cough: recurrent, harsh, croup-like cough
 Wheezing and/or stridor on expiration
 Recurrent respiratory infections and sputum production
Extrathoracic lesion
 Inspiratory stridor
 Recurrent respiratory infections and sputum production
Differential diagnosis of upper airway obstruction
 Infectious: parainfluenza virus, bacterial membranous tracheitis, tuberculous tracheitis,
rhinoscleroma
 Non-infectious: primary pulmonary amyloidosis, relapsing polychondritis, Wegener’s
granulomatosis
 Other tracheal diseases: tracheal stenosis, bronchogenic cyst, intrinsic
tracheal/bronchial obstruction, tracheoesophageal fistula.
 Pediatric entities: tracheal/bronchial atresia, tracheal bronchus.
Diagnosis
Requires demonstration of dynamic assessment of the trachea and bronchi throughout the
respiratory cycle with demonstration of airway collapse in expiration.
 Tracheobronchography
o Preferred method of diagnosis because of ability to assess the airways
dynamically and easy to perform in intubated patients.
 Bronchoscopy
o Of limited value because the airway may be splinted by the bronchoscope, which
will reduce the dynamic compression of the airway
o May not assess small airways beyond a stenosis
 Non-contrast fluoroscopy
o Can assess the trachea well, but does not show bronchi adequately
 CT/MRI
o Spiral and ultrafast CT and MRI with rapid acquisition sequences
o Capture trachea and main bronchi, but do not reliably capture lesions because
the airways move in and out of the plane of imaging during respiration
Beth Israel Deaconess Medical Center Residents’ Report
Categorization
 Type 1: Intrinsic defect/immature forms of the cartilaginous portion of the trachea. Leads
to increased proportion of membranous trachea. (also called primary malacia)
 Type 2: Extrinsic tracheal compression by cardiovascular structures, tumors, lymph
nodes, or other masses. Can be congenital or acquired. (aka secondary malacia)
 Type 3: Result from prolonged positive pressure ventilation or infectious/inflammatory
process that compromises the intrinsic cartilaginous support of the trachea. Leads to
degenerationof previously normal cartilage. (also a form of secondary malacia).
Treatment
Steroids and bronchodilators
 May improve peripheral airway obstructive disease and reduce dynamic compression of
airways
Stents
 Expanding wire stents
o Placed via bronchoscopy and balloon-expandable angioplasty
o May cause granulomata formation, severe hemoptysis, or tracheobronchial
rupture
 Dumon’s dedicated tracheobronchial stents
o Made of molded silicon to reduce granulation tissue formation
o Used more for tracheal obstruction secondary to tumors, but not well suited for
diffuse tracheobronchomalacia because of stent migration and secretion
retention.
 Y-stent
o Useful for diffuse disease in the tracheobronchial tree
Surgery/Tracheoplasty
 Reserved for patients in whom medical therapy has failed and underlying causes (tumor,
etc) have been removed.
References
Braman S, Grillo H, Mark EJ. 44 year old man with tracheal narrowing and respiratory stridor. NEJM
1999;341(17):1292-1299.
Burden RJ, Shann F, Butt W, et al. Tracheobronchial malacia and stenosis in children in intensive care:
bronchograms help to predict outcome. Thorax 1999;54(6):511-517.
Collard P, Freitag L, Reynaert MS, et al. Respiratory failure due to tracheobronchomalacia. Thorax 1996;51(2):224226.
Furman RH, Backer CL, Dunham ME., et al. The use of baloon-expandable metallic stents in the treatment of
pediatric tracheomalacia and bronchomalacia. Archives of Otolaryngology Head and Neck Surgery.
1999;125(2):203-207.
Spittle N, McClusky A. Tracheal stenosis after intubation. BMJ 2000;321(7267):1000-1002.
UpToDate
Beth Israel Deaconess Medical Center Residents’ Report
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