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Managing the Complexities of Congenital Diaphragmatic Hernia
Congenital diaphragmatic hernia (CDH) is a complex and challenging anomaly in which attention to every tiny detail is crucial to achieving the best outcomes. In this series, staff from the Center for Fetal Diagnosis and Treatment present a full overview of CDH, the comprehensive care required to manage the condition, and the ongoing research that holds promise for affected babies.
Videos in this Series
Transcript: Introduction
Antoinette Spence, parent: We were going for our 20-week ultrasound, which was the last ultrasound of the pregnancy.
Anthony Spence, parent: We had decided to find out whether it was a boy or a girl.
Antoinette Spence: We were so excited that we found out that she was girl that we didn't notice that the ultrasound technician kept going over the same spot.
Anthony Spence: The doctor told us that there was a problem.
Antoinette Spence: -- a hole in Simone's diaphragm.
Anthony Spence: -- a very serious condition --
Antoinette Spence: My husband often describes that he felt his heart drop at that moment when we found out that she had to have surgery and had this complicated illness that we had never even heard out of.
Holly L. Hedrik, MD: Congenital diaphragmatic hernia is a birth defect. It's a birth defect that happens very early in gestation.
Mark P. Johnson, MD: The diaphragm doesn't form properly between 9 and 10 weeks of gestational age.
N. Scott Adzick, MD: There's a hole in the diaphragm muscle, usually on the left side, that has catastrophic effects before birth.
Holly L Hedrik, MD: An event happens where abdominal contents move into the chest.
N. Scott Adzick, MD: The intestine and other organs in the abdomen can go up through this defect, into the chest, and compress the developing lungs.
Mark P. Johnson, MD: Because there's so much other tissue in the chest, the lungs can't expand and grow and develop normal.
N. Scott Adzick, MD: It's of little consequence before birth for the baby getting oxygen and that sort of thing, but the real road test begins at the time of birth when the umbilical cord is clamped, and those small lungs have to work.
Natalie E. Rintoul, MD: The problem starts with the hole in the diaphragm, but that's not the whole story. Really, a lot of times what we struggle with are the the pulmonary vessels. The pulmonary vasculature is very small.
N. Scott Adzick, MD: You can imagine a tree with a trunk and branches and twigs all the way out to the periphery of the tree. In babies with diaphragmatic hernia, not only is the room for the tree small, but the tree is very pruned so there are many, many fewer vessels per cross-sectional area of lung. And those blood vessels that are present are abnormal in that they are -- they have enhanced or increased muscularization that tend to make them pinch down, clamp down.
Marcus Davey, PhD: When this muscle constricts, it actually decreases the lumen of the vessel and reduces blood flow to the lung.
N. Scott Adzick, MD: As the heart after birth tries to pump blood through the lungs, it has a difficult time and there's pulmonary hypertension, high blood pressure in the pulmonary circulation.
Holly L. Hedrik, MD: And that ends up being, in most cases, the single most important factor in determining whether or not they can survive.
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