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Congenital Pulmonary Airway Malformation (CPAM), previously called congenital cystic adenomatoid malformation (CCAM) is a developmental anomaly of the lung characterized by abnormal branching of terminal bronchioles, resulting in nonfunctioning, cystic or solid lung tissue that lacks normal alveoli and has no normal airway connection.
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Congenital Pulmonary Airway Malformation - causes, symptoms, diagnosis, treatment, pathology
Modified Stocker CPAM classification:
| Type | Cyst Size | Frequency | Features |
|---|---|---|---|
| Type 0 | Tracheobronchial origin | 1-3% | Rare; small firm lungs, incompatible with life |
| Type 1 | Macrocystic (>2 cm) | 60-70% | Most common (~60%); few large cysts; best prognosis |
| Type 2 | Medium cysts (<2 cm) | 15-20% | Multiple small cysts; often associated with other anomalies |
| Type 3 | Microcystic/solid | 5-10% | Bulky, echogenic mass; poor prognosis; can cause mediastinal shift |
| Type 4 | Large peripheral cysts | 10-15% | May mimic pleuropulmonary blastoma (PPB); originates from distal acinus |
Stocker JT. Congenital pulmonary airway malformation: a new name for an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology 2002; 41: 424–458.