Prevalence:

• 1 in 15,000 births

Ultrasound diagnosis:

• 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.

Associated abnormalities:

• 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.

Fetal therapy:

• Ultrasound guided laser coagulation of the feeding vessel in cases of severe hydrothorax or hydrops.

Follow up:

• 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.

Prognosis:

• Survival: >95%.
• Postnatal therapy: endoscopic removal of mass or selective embolization of the feeding artery.

Recurrence:

• No increased risk of recurrence.