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  • Congenital Diaphragmatic Hernia (CHD)

  • Chest Graph
    Figure 1: Normal chest anatomy.
     CDH Graph

    Figure 2: Congenital diaphragmatic hernia. Intestinal loops
    exist in the left chest cavity, which pushes the heart toward
    the right lung and compresseses the left lung.

    What is a congenital diaphragmatic hernia (CDH)?

    Congenital diaphragmatic hernia (CDH) refers to a defect in the diaphragm that allows the abdominal organs (stomach, intestine, liver, spleen) to move into the chest cavity. CDH can occur on either the left or the right side but is most common on the left.

    Why worry about a diaphragmatic hernia?

    Babies who have CDH suffer from small and underdeveloped lungs. This condition is termed pulmonary hypoplasia. CDH allows the intestines to move up into the chest cavity. This prevents the heart and the lungs from developing properly (figures 1 and 2).

    CDH is a life-threatening illness. When the lungs of a baby do not develop properly during pregnancy it can be difficult for the baby to breathe after delivery. The lungs of babies born with CDH are hypoplastic. The alveoli (small respiratory pouches) that are present are much more fragile and are missing surfactant (a lubricating liquid found in the lungs). In comparison, normal lungs at birth have millions of small alveoli, which can best be thought of as many, many clusters of grapes. When babies are born with CDH and hypoplastic lungs, there are not enough alveoli to get oxygen in the blood, or to get rid of carbon dioxide from the body. When this occurs, the lack of oxygen leads to severe illness requiring very aggressive supportive measures.

    CDH is also of concern because of the possibility of associated birth defects (anomalies) that may impact on the baby's survival. In some cases the heart may have defects. The gastrointestinal tract (the esophagus, stomach and intestines) may be malfunctioning because they developed in the chest, rather than in the abdomen. Kidney and central nervous system defects also are known to occur in babies with CDH. Finally, chromosomal defects (most commonly trisomy 21 (Down syndrome), 18, and 13) are found in approximately 10 to 30 percent of babies with CDH.

     

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    The Fetal Treatment Program is a partnership of Hasbro Children's Hospital, Women & Infants' Hospital and The Warren Alpert Medical School of Brown University.