Treatments for Cystic Lung Disease in Children

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What treatments are available for cystic lung disease?

Bronchogenic cysts

Since a cyst may compress your child's airway, it is important that it be removed in a careful and controlled manner.  Depending on your child's age, symptoms, and the location of the cyst, it may be possible to remove it via thoracoscopic (minimally invasive) surgery.

To help your child during surgery, the surgeon may include careful ventilation or even use extracorporeal membrane oxygenation (ECMO). This is a machine that takes over for the lungs, providing oxygen and respiratory support.

Even if your child isn't experiencing symptoms from the bronchogenic cyst, it should be removed to prevent long-term complications, including infection, bleeding or malignant degeneration.

CCAMs

There are several ways to treat symptomatic CCAMs. These include:

  • surgical aspiration (fluid suctioning) of a cystic lesion
  • removal of a solid mass or affected lobe during fetal

For lesions that seem to be regressing and are not producing further symptoms, your child's doctors will perform simple post-natal follow up, which will include careful chest imaging and potential delayed pulmonary resection.

Though this course of action is open to debate, some people choose to have these asymptomatic CCAMs removed because of the rare but reported cases of malignant degeneration in these lesions as the babies grow into adults.

A CCAM may also produce neonatal respiratory distress complicated by pulmonary hypertension, and such babies may require urgent neonatal operation and/or aggressive management of their acute respiratory failure including the use of extracorporeal membrane oxygenation (ECMO), a machine that takes over for the lungs, providing oxygen and respiratory support.

Lobar emphysema and pulmonary sequestration

For these conditions, surgery is almost always required to remove the affected lobe (called a lobectomy) in order to prevent further damage to the entire lung. If done promptly, surgical treatment is almost always successful, leaving little or no permanent impairment of breathing capability.

What is the long-term outlook for a child with a cystic lung disease?

Fortunately, children typically do well after the treatment of their cystic lung disease. If they have severe associated anomalies or if the normal lung development has been compromised, then the outcomes may be more limited.

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- Sandra L. Fenwick, President and CEO

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