Lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue

• Communicates with airway bronchus unlike pulmonary sequestration
• This is secondary to bronchiole tissue overgrowth!

Sx: newborns respiratory distress, older children infections

Rapid decompensation can occur with ventilator → needs emergent resection of cystic structure

Risk of malignancy

Dx: in utero can measure CAM volume ration (CVR) = volume of lung/head circumference. If > 1.6 high risk for fetal hydrops and will need biweekly US and will need to give steroids to decrease size

Tx: Lobectomy