Pulmonary Sequestration

By: Veronica

Pulmonary Sequestration Explained

Pulmonary Sequestration is defined as a mass of tissue that develops inside the lungs, but has no respiratory function. It does not assist in gas exchange! The word sequestrate means "to separate". The term bronchopulmonary sequestration is the term that is given to a lung region parenchyma that is completely or partially separated from the bronchopulmonary tree of the lungs. It is a rare congenital disorder that happens in 0.1% of human beings, occurs more often in males than females (4:1) and children and unborn babies. It is unusual and very uncommon cystic mass. It is not life threatening, but can cause many complications such as cardiovascular issues, lung infections like TB and Bronchial cancer. Fatal if blood vessels inside the lungs begin to hemorrhage.
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Blood supply

The sequestrations do not get blood supply from the lungs, but rather its own artery and its own vessel. This is why it can be fatal to the patient when trying to remove it.
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2 types of Sequestrations

1. Intralobar Sequestrations this is the majority of cases 75%-85%. These show up later in childhood with infections that are often reoccurring. These occur within the existing membrane or the visceral pleura of the lungs. Also presents in adults after the age of 20, most patients are asymptomatic and have these abnormalities for years and are only diagnosed during routine chest x-rays for unrelated issues.

2. Extralobar Sequestrations are less common approximately 15%-20%. These usually present in the neonate with respiratory distress, cyanosis and or infection. This type is completely enclosed in their own pleural sac. These are common in neonates and unborn babies that are likely to develop other malformations like heart defects and GI deformities.

Prevention

There is really nothing anyone can do to prevent this from happening as its congenital.
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Theraputic Interventions

There is no medications to take for this disorder. The only thing that has proven effective is the complete removal of the sequestration via resection or surgery. The surgeons will go in and clamp off the artery and vein that feeds the sequestration and remove it. The sequestrations can be seen via X-Ray, Ultrasound, and CT scan. In cases where surgery is to great angiographic emboization at the feeding systemic vessels are used. This therapy has proven successful in the definitive management if intralobar sequestration and is not commonly used as an alternative treatment for children. Intalobar sequestrations tend to be more difficult because of their location in the visceral pleura of the lung which may result in some lung tissue loss during surgery.

New Research

The new testing that is being used is the Aortography which uses ultrasound to measure blood flow to the sequestration. It is used to determine the arteries supplying the blood to the sequestration and the flow from the sequestration back to the heart.

Questions?

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