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What causes bronchopulmonary sequestration?

What causes bronchopulmonary sequestration?

The cause of bronchopulmonary sequestration remains unknown. It has not been linked to a genetic or chromosomal anomaly, and does not appear to run in families (is not hereditary). Most clinicians believe the condition begins during prenatal development when an extra lung bud forms and migrates with the esophagus.

What is lung sequestration?

Pulmonary sequestration is a condition in which a segment or lobe of dysplastic lung tissue exists with no communication with the rest of the tracheobronchial tree and receives an anomalous systemic vascular supply, separate from the rest of the lung. It is, therefore, a nonfunctional tissue.

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.

Can CPAM go away?

If the CPAM grows, which will occur in a small number of cases, it can cause increasing problems for you and/or the baby, including the development of hydrops. Most lesions will either stay the same size or actually decrease in size or disappear as the pregnancy progresses.

Is CPAM serious?

In about 10 percent of cases, CPAM can cause serious problems for the baby and sometimes, for the mother as well. The most serious problems occur when babies develop hydrops, a prenatal form of heart failure characterized by fluid build-up in the skin, chest or abdomen.

Does CPAM require surgery?

Most babies with a small CPAM are born with no symptoms and can go home after a few days in the hospital. Surgery to remove the CPAM is usually performed when a baby is three to six months old. This is done through an operation in the chest, and the part of the lung that contains the CPAM is removed.

Is CPAM curable?

To treat these complications, doctors may use a ventilator to help the baby breathe. Newborns with very large lesions may need to have surgery immediately after birth to remove the CPAM and prevent breathing problems. In some cases, the CPAM can be removed at birth with a surgery called the EXIT procedure.

Can you live with CPAM?

Babies who have CPAM and hydrops typically will not survive unless they can respond quickly to medicine or undergo fetal surgery to remove the lesions. Hydrops can cause “mirror syndrome,” in which the mother “mirrors” the baby’s illness. The mother can develop high blood pressure and fluid retention.