What Is Congenital Diaphragmatic Hernia: Illustrated

View an illustrated explanation of postnatal surgery to repair congenital diaphragmatic hernia (CDH).

What is congenital diaphragmatic hernia? 0:03
How does congenital diaphragmatic hernia impact lung development? 0:31
How is congenital diaphragmatic hernia diagnosed? 0:50
What predicts the outcome in congenital diaphragmatic hernia? 1:05
What is pulmonary hypoplasia? 1:12​
What is pulmonary hypertension? 1:42
How is congenital diaphragmatic hernia surgically repaired after birth? 1:55
Why is it important to follow babies born with congenital diaphragmatic hernia over the long term? 2:28


What Is Congenital Diaphragmatic Hernia: Illustrated

Holly L. Hedrick, MD, FACS: Congenital diaphragmatic hernia or CDH is a rare condition in which a defect in the diaphragm allows the abdominal organs to move into the chest during fetal development. The diaphragm is a muscle that separates the heart and lungs in the chest from the liver, stomach, intestines and other organs in the abdomen. With CDH, as the baby develops in the womb, the diaphragm doesn’t close properly. The defect in the muscle may be on the left or right side and small or large. If the hole is large enough, the intestines, stomach, spleen, and liver can move into the chest. There, they can crowd the heart and lungs and keep the lungs from growing normally. Because it is a large heavy organ, the liver causes particular problems.

Most of the time, CDH is diagnosed during pregnancy. At Children’s Hospital of Philadelphia, we use sophisticated imaging to look at all aspects of the fetus. Liver position and lung size are two of the predictors of how the baby will do.

Many babies with CDH develop a condition called pulmonary hyperplasia, which means small lungs. When they are born, they will have trouble breathing. At our hospital, the babies are born in our special delivery unit built specifically for babies with birth defects. They’re handed to a waiting team of doctors and nurses, stabilized and taken to our newborn infant intensive care unit close by. Many babies with CDH develop high blood pressure in the lungs called pulmonary hypertension.

We monitor and treat this condition with cardiologists specializing in pulmonary hypertension. Once the baby stabilizes, it is time for surgery to repair the diaphragm. The anesthesiologist and surgeons perform the operation at bedside in the NICU. The baby is placed under general anesthesia. The surgeons make an incision below the ribs, they gently bring the liver, stomach and intestines into place. They then repair the diaphragm using stitches and often a Gore-Tex patch. The baby will recover in the NICU for weeks to months. As babies grow, their brains and lungs grow as well. In fact, the child’s lungs will continue to grow for several years.

At Children’s Hospital of Philadelphia, we treat more babies with CDH than any other hospital in the world. Our team follows these children into adulthood, providing interdisciplinary long term follow-up care.

We also have a large research program so we can learn more about CDH and find new ways to treat it. While it is a very serious birth defect, many babies with CDH go on to thrive, each in their own way.


Topics Covered: Congenital Diaphragmatic Hernia (CDH)

Related Centers and Programs: Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment

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