Congenital Lung Malformations: CPAM, BPS, CLE, and Bronchogenic Cyst Explained
hosted by Dr. Jill Knepprath
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
CPAM (congenital pulmonary airway malformation) is the most common congenital lung malformation.
CPAM is an overgrowth of the terminal bronchioles.
CPAM is most commonly found in just one lobe, usually the lower lobe.
CPAM has a direct communication with the lung parenchyma and the blood supply.
Bronchopulmonary sequestration doesn't have a continuation with the tracheobronchial tree.
Bronchopulmonary sequestration has an aberrant systemic artery supplying it, usually coming off of the aorta.
Bronchopulmonary sequestrations can be intralobar or extralobar.
Intralobar bronchopulmonary sequestrations are within the visceral pleura.
Intralobar bronchopulmonary sequestrations, even though not aerated, get some collateral ventilation from surrounding lung tissue and are prone to infection.
Intralobar bronchopulmonary sequestrations are usually in the lower lobes.
Extralobar bronchopulmonary sequestrations are less common than intralobar.
Extralobar bronchopulmonary sequestrations have their own pleura.
Infections are less common in extralobar bronchopulmonary sequestrations.
Extralobar bronchopulmonary sequestrations can be under the diaphragm.
Congenital lobar emphysema is a focal cartilaginous abnormality of the bronchial wall which creates a valve effect.
Congenital lobar emphysema causes the subsequent lobe to overinflate.
Congenital lobar emphysema only affects one lobe.
The left upper lobe and the right middle lobe are the most commonly affected in congenital lobar emphysema.
Bronchogenic cyst is a unilocular malformation.
Bronchogenic cyst happens because of an abnormal budding of the primitive ventral foregut.
Bronchogenic cyst contains cartilaginous tissue, smooth muscle, and bronchial glands.
Bronchogenic cyst is usually in the mediastinum next to the trachea or the main stem bronchi.
Bronchogenic cyst does not communicate with the tracheobronchial tree unless it's a rarer intrapulmonary form.