KidsMD Health Topics

Cystic Lung Disease in Children

  • Cystic lung disease is a term used to describe four distinct conditions that may share a degree of common origin during fetal development. These conditions are:

    Bronchogenic cysts (also called a bronchopulmonary foregut malformation)

    • This is a cyst usually found on midline chest structures of the body, such as the esophagus and trachea.
    • They are sometimes also found on the lower lobes (sections) of the lung.
    • Some of these cysts may become infected or grow so large that they compromise the function of the adjacent airway.

    Cystic adenomatoid malformation (CCAM)

    • This is a benign (non-cancerous) mass of abnormal lung tissue, located usually on one lobe of the lung.
    • This condition is caused by overgrowth of abnormal lung tissue that may form fluid filled cysts or result in the failure of the development of the tiny air sacs that characterize a normal lung. The tissue does not function as normal lung tissue.
    • There are two types of CCAMs: Type I is distinguished by one or more large cysts, and type II has both solid regions and cysts.

    Lobar emphysema

    • This is a condition that involves an obstruction in a lobe of lung that causes airflow to become trapped during inflation and expiration of the lungs.
    • It may be congenital (present at birth) or acquired (not present at birth).
    • It may affect only a part, but more often the entire lobe is involved, usually the left upper lobe.
    • The obstruction of air flow causes the lung to distend (stretch out and expand). Depending on the extent of distension, the affected lobe can compress the unaffected lobe below or above it, and/or the opposite lung by extending across the mediastinum (the area that separates the lungs and houses vital structures such as the heart and major vessels).
    • Without treatment, this condition can cause acute respiratory distress and even circulatory collapse.

    Pulmonary sequestration

    • This is a mass of nonfunctioning pulmonary (pertaining to the lungs and respiratory system) tissue.
    • This kind of mass is subdivided into two types: extralobar or intralobar.
      • An extralobar sequestration is typically located just above or just below the diaphragm. It is separated from the lung and enveloped by its own pleura (membrane that covers the outside of the lung).
      • An intralobar sequestration is typically found in the lower lung lobes, more often on the left side, and is characterized by recurrent respiratory infections.
    • Sequestrations can interfere with normal circulation, cause respiratory infections and/or lead to heart failure.

    The Center for Healthy Infant Lung Development (CHILD) at Boston Children's Hospital cares for newborns, infants and children with many different forms of lung disease. Program expertise ranges from care of premature infants to care of full-term infants with breathing difficulties.

    We offer a comprehensive, team-based approach to maximize the growth and development of the lungs, which helps children reach their maximum potential in all aspects of their normal activities, including exercise, feeding and learning. Our staff is available 24 hours per day, 7 days per week, and we pride ourselves in how available we are to our families.

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