Congenital Pulmonary Airway Malformation

  • Etiology: hamartoma
  • CXR: retention of fluid at birth and air trapping later
  • Imaging: can be in any lobe, Type I (65%) has multiple large cysts, Type II (20%) has variable smaller cysts and can be associated with sequestration = hybrid lesion, Type III (<10%) has many tiny cysts that can appear solid, Type 4 (10%) has large cysts with mass effect
  • DDX: pneumatocoele – pneumatocoele should decrease in size over time, CPAM increases in size over time
  • Treatment: surgical due to low potential for malignancy

Cases of Congenital Pulmonary Airway Malformation

CXR of congenital pulmonary airway malformation
CXR shows the left hemithorax filled with multiple cysts of various sizes causing mediastinal shift to the right. There is herniation of some normal appearing left apical lung across the midline. The stomach is in the abdomen.
Pathological image of congenital pulmonary airway malformation / CPAM / CCAM / congenital cystic adenomatoid malformation
Pathological image shows multiple cystic lesions in the right upper lobe.