- 1 in 15,000 births
- Hyperechogenic mass in the lung, mostly in the left lower lobe.
- Color Doppler demonstrates a feeding vessel that arises from the descending aorta.
- In 75% of cases it is intralobar, making it indistinguishable in appearance from microcystic CPAM.
- In 25% of cases it is extralobar, located outside the normal lung with its own visceral pleura; in most of these cases there is an associated pleural effusion.
- The incidence of chromosomal abnormalities and genetic syndromes is not increased.
- Defects in other systems, mainly diaphragmatic hernia and cardiac or vertebral anomalies are found in up to 50% of cases with extralobar sequestration.
- Ultrasound guided laser coagulation of the feeding vessel in cases of severe hydrothorax or hydrops.
- Ultrasound scans every 4 weeks to monitor growth of the tumor and hydrothorax or hydrops.
- In >30% of cases there is regression or disappearance of the tumor during the 3rd trimester.
Timing and route of delivery:
- Place: hospital with neonatal intensive care and pediatric surgery.
- Time: 38 weeks.
- Method: induction of labor aiming for vaginal delivery.
- Survival: >95%.
- Postnatal therapy: endoscopic removal of mass or selective embolization of the feeding artery.
- No increased risk of recurrence.