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Congenital Diaphragmatic (di-a-frag-mat-ic) Hernia
Diagnosis
Diagnosis is usually made during a prenatal ultrasound by the obstetrician. In addition, a fetal MRI might be done to learn more about the defect.
Cause
The exact cause of congenital diaphragmatic hernia is unknown. During fetal (before birth) development the muscles in the diaphragm do not grow together, leaving a hole. The hole allows the intestines to move up into the chest causing the heart to be slightly pushed to one side. 80% of the holes in the diaphragm occur on the left side of the chest. The lungs do not get a chance to grow normally, causing difficulty breathing at birth. Diaphragmatic hernia is a life-threatening emergency if not treated. Studies show that 1 in every 4,000 babies will be born with this problem and between 50-75 % of babies born with diaphragmatic hernia survive.
Treatment
Surgery needs to be done to repair the diaphragmatic hernia, but the time of surgery varies. The surgery is not an emergency. However, breathing for the baby is usually an emergency immediately after birth. Babies with diaphragmatic hernia have small lungs and can have high pressure in the lungs. This causes the baby to need a lot of help breathing using a ventilator or breathing machine. Nitric oxide is a medicine that may also be used to help make the pressure in the lungs lower. If the lungs are terribly damaged and the baby is not getting any better, the baby may require a heart lung bypass machine, called ECMO.
Surgery
Surgery will be done once the baby’s breathing is stable. It may be days or weeks before surgery can be done. In the operating room, the pediatric surgeon will make an incision in the abdomen just below the rib cage. The surgeon will push the intestines and other organs back into the belly and close the opening in the diaphragm. Sometimes, if the hole is too big and the surgeon can’t sew the diaphragm together, the surgeon may use an artificial patch (such as Gortex).
Hospitalization
A baby with diaphragmatic hernia will likely be in the hospital for quite a while after surgery, recovering and learning to feed.