Congenital diaphragmatic hernia, or CDH, is a condition that develops before birth. Babies with CDH have a hole in their diaphragm, the flat muscle that separates the chest from the abdomen. Normally, there is a hole in the diaphragm early in pregnancy, which closes by the end of the third month of the pregnancy. In CDH, the hole doesn't close properly. CDH occurs in about one in every 2,500 births.
When a baby has CDH, some of the organs in the abdomen, including the stomach, intestine, liver, spleen and kidneys, may move into the chest. As a result, the lungs can't grow to normal size, causing a condition called pulmonary hypoplasia. The majority of these defects occur on the left side.
While in the uterus, a fetus doesn't need lungs to breathe because oxygen is delivered through the mother's placenta. However, once the baby is born healthy lungs are necessary. If the lungs haven't developed normally and are too small, the baby won't get enough oxygen to survive.
Most babies with CDH can be treated successfully after birth, although a few with the most severe form of this condition may be treated while in the womb. The UCSF Fetal Treatment Center was the first facility in the world to offer treatment for congenital diaphragmatic hernia while a baby is still in the uterus.
Symptoms of congenital diaphragmatic hernia may include:
CDH is usually diagnosed before birth using an ultrasound, a non-invasive procedure in which sound waves form a picture of the fetus. An ultrasound also allows doctors to determine the severity of the defect.
The two factors used to grade the severity of CDH are the position of the liver and the lung-to-head ratio. Liver position refers to whether or not any portion of the liver has herniated, or moved up into the chest. Fetuses with a portion of the liver in the chest have a more severe condition and a low survival rate. The lung-to-head ratio is a numeric estimate of the size of the lungs based on measuring the amount of visible lung. High lung-to-head ratios are associated with good outcomes.
Examination of your baby after birth may include:
Most babies with CDH are born with such small lungs that they can't breathe on their own. If this happens your baby will be moved to the Intensive Care Nursery, where a mechanical ventilator — a breathing machine — will help your baby receive enough oxygen. An extracorporeal membrane oxygenation (ECMO) machine, which temporarily does the work of breathing for your baby, may also be recommended. In ECMO, blood is drained from your baby into an artificial lung where oxygen is added and carbon dioxide is removed, then the blood is pumped back into your child.
For babies whose liver hasn't grown into the chest cavity, survival with treatment after birth is very good, about 90 percent. For these infants, we recommend that the baby be born in a hospital with an Intensive Care Nursery that has the capability to provide ECMO. ECMO treatment can be used only for a limited time, usually about two weeks.
Reviewed by health care specialists at UCSF Benioff Children's Hospital.
Last updated
June 17, 2010
Intensive Care Nursery
505 Parnassus Ave., Fifteenth Floor
San Francisco, CA 94143-0210
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Fax: (415) 353-1202
Fetal Treatment Center
400 Parnassus Ave., A123
San Francisco, CA 94143
Phone: (415) 476-0445
Fax: (415) 502-0660
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Congenital Anomalies Follow-up Program
400 Parnassus Ave., Suite A123
San Francisco, CA 94143
Phone: (415) 476-9717
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