(aka Tracheobronchomegaly)
Epidemiology
- First reported in 1932
- Probably congenital (familial clustering reported)
- Sex: more common in men
- Age: usually presents in 20 s-30 s
Physiology
- Congenital abnormality of the trachea and main bronchi that is characterized by atrophy or absence of elastic fibers and thinning of the smooth muscle layer
- Flaccid Airways: show marked collapsibility during a forced expiration or during a cough
- Ineffective cough mechanism: causes retention of mucus, chronic colonization with bacteria, and recurrent pneumonias and eventual bronchiectasis
Diagnosis
- FOB: striking dilation of intrathoracic trachea (mean diameter >31 mm) and major bronchi (mean diameter of right MSB >24 mm/left MSB >23 mm)
- Flaccid Airways: which collpase during cough
- CXR/Chest CT Pattern:
- Redundant muculomembranous tissue between cartilaginous rings appear like multiple diverticuli along lateral trachea
Clinical
- Severe Recurrent or Chronic Pulmonary Infection:
- Bronchiectasis (see [[Bronchiectasis]])
Treatment
- Treat Bronchiectasis: standard therapy
- Bronchodilators: no documented therapeutic effect
References
- xxx