The radiograph shows a large lucency in the left lung with shift to the right. The CT shows multiple cystic areas in the left upper lobe and lingula with volume expansion and shift to the right. The majority present in the neonatal period. Often fetal ultrasound is able to diagnose this condition. Macrocystic disease has a better prognosis than microcystic disease, which often presents with hydrops and lung hypoplasia.
Treatment is with resection with a good prognosis.