Congenital pulmonary sequestration
Routine antenatal 2nd trimester ultrasound
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A single viable fetus was seen in cephalic presentation.
A large wedge shaped echogenic shadow was seen in the lower fetal left chest, pushing the cardia contralaterally. No internal cystic shadows were seen. A color Doppler sonogram demonstrated an ectopic blood supply to the mass from the thoracic aorta - diameter of the supplying vessel was 3.4mm.
- Bronchopulmonary sequestration (BPS) is a rare congenital malformation of the lower respiratory tract.
- It consists of a nonfunctioning mass of normal lung tissue that lacks normal communication with the tracheobronchial tree, and that receives its arterial blood supply from the systemic circulation.
- BPS is estimated to comprise 0.15 to 6.4 percent of all congenital pulmonary malformations, making it an extremely rare disorder.
- Sequestrations are classified anatomically:
- Intralobar sequestration (ILS) in which the lesion is located within a normal lobe and lacks its own visceral pleura.
- Extralobar sequestration (ELS) in which the mass is located outside the normal lung and has its own visceral pleura
- The blood supply of 75% of pulmonary sequestrations is derived from the thoracic or abdominal aorta.
- The remaining 25% of sequestrations receive their blood flow from the subclavian, intercostal, pulmonary, pericardiophrenic, innominate, internal mammary, celiac, splenic, or renal arteries.