What is Extralobar pulmonary sequestration?
Pulmonary sequestration. Extrapulmonary sequestration. Computed tomography. Pulmonary sequestration is a rare congenital malformation in which a segment of nonfunctional lung lacks a bronchial connection and possesses an aberrant blood supply. Most often, blood supply is from the infradiaphragmatic aorta.
Is pulmonary sequestration life threatening?
While it is not in itself a life-threatening condition, a pulmonary sequestration can cause health complications including cardiovascular problems, long-term infections like tuberculosis, and bronchial cancer. It could be fatal if blood vessels in the lung begin to hemorrhage.
What is Bronchipulomonary sequestration?
Bronchopulmonary sequestration (BPS) is a rare malformation of the lung that occurs before a baby is born. With BPS, a piece of lung tissue develops without being connected to the airways, sometimes inside the lung and sometimes outside of it.
Is CPAM hereditary?
There is no known cause for CPAM, which was previously referred to as congenital cystic adenomatoid malformation (CCAM). The condition is relatively rare, affecting about 1 in 25,000 pregnancies. It is slightly more common in males than in females. CPAM is not hereditary, so it usually does not recur in families.
What is Pulmonary sequestration?
Pulmonary sequestration (PS) is a congenital lung malformation that consists of a nonfunctioning lung tissue with no apparent communication with the tracheobronchial tree [1]. The blood supply to PS is through aberrant vessels from systemic circulation, most commonly the descending thoracic aorta.
When to know if you have extralobar pulmonary sequestration?
Extralobar pulmonary sequestration (ELS) is a subtype of pulmonary sequestration, the other type being intralobar pulmonary sequestration (ILS). It is usually encountered in infants, most being diagnosed before six months.
When to use Intralobar or extralobar sequestration?
intralobar sequestration (ILS) accounts for the majority (75-85% of all sequestrations 4,5,7) present later in childhood with recurrent infections. extralobar sequestration (ELS) less common (15-25% of all sequestrations 4,5,7) usually present in the neonatal period with respiratory distress, cyanosis, or infection.
Where does the tissue for pulmonary sequestration come from?
Pulmonary sequestration is an uncommon congenital abnormality in which nonfunctioning lung tissue is supplied by an anomalous systemic artery. Both the extralobar and intralobar forms probably develop from an accessory lung bud from the primitive foregut.