What is the most common form of pulmonary sequestration?
Pulmonary sequestration represents approximately 6% of all congenital pulmonary malformations. Intrapulmonary sequestrations are the most common form, and 60% of these are found in the posterior basal segment of the left lower lobe. Overall, 98% occur in the lower lobes.
What is Extralobar 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.
What does lung sequestration mean?
Pulmonary sequestration is a rare congenital (present from birth) malformation where non-functioning lung tissue is separated from the rest of the lung and supplied with blood from an unusual source, often an artery from systemic circulation.
What is Bronchipulomonary sequestration?
Bronchopulmonary sequestration, also known as BPS or pulmonary sequestration, is a rare birth defect in which an abnormal mass of nonfunctioning lung tissue forms during prenatal development. It can form outside (extralobar) or inside (intralobar) the lungs, but is not connected directly to the airways.
How common is pulmonary sequestration?
Lung sequestration is very rare and only represents 0.15–6.4% of congenital pulmonary abnormalities . The most important presenting symptoms are recurrent pulmonary infections or productive cough. Severe hemoptysis as the first symptomatic manifestation has been reported very rarely .
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.
Is Pulmonary sequestration serious?
How is pulmonary sequestration diagnosed?
Pulmonary sequestrations are diagnosed with a prenatal ultrasound showing a mass in the chest of the fetus. The mass may displace the heart from its normal position or push the diaphragm downward, but the key feature of a sequestration is the artery leading from the cystic mass directly to the aorta.
What is a hypoplastic lung?
Pulmonary hypoplasia is a condition in which the lungs are abnormally small, and do not have enough tissue and blood flow to allow the baby to breathe on his or her own. This can be a life-threatening condition.
Is pulmonary sequestration serious?
What is the life expectancy of someone with bronchiectasis?
Most people diagnosed with bronchiectasis have a normal life expectancy with treatment tailored to their needs. Some adults with bronchiectasis developed symptoms when they were children and live with bronchiectasis for many years. Some people, who have very severe bronchiectasis, may have a shorter life expectancy.
What kind of resection is needed for pulmonary sequestration?
Traditionally treatment has been a surgical resection. Extralobar sequestrations with their separate pleural investments can usually be removed sparing normal lung tissue, although, with an intralobar type, segmental resection or even lobectomy will be necessary.
How is pulmonary sequestration similar to the bronchial tree?
The two types of sequestration are similar in their relationship to the bronchial tree and arterial supply/venous drainage but differ in their relationship to the pleura. By definition, there is no communication with the tracheobronchial tree.
What kind of disease is associated with extralobar sequestration?
Extralobar sequestrations almost always affect the left lower lobe, however, ~10% of extralobar sequestrations can be subdiaphragmatic 8. Associated disease is common with the extralobar type (50-60%):
How does pulmonary sequestration affect the lower lobe?
Almost all cases occur sporadically. Overall, sequestration preferentially affects the lower lobes. 60% of intralobar sequestrations affect the left lower lobe, and 40% the right lower lobe. Extralobar sequestrations almost always affect the left lower lobe, however, ~10% of extralobar sequestrations can be subdiaphragmatic 8.