Congenital Diaphragmatic Hernia
Congenital diaphragmatic hernia (CDH) is a condition where there is a hole in the diaphragm and organs from the abdomen go up into the chest cavity. CDH was described in 1848 and the first repair in a baby was by Robert Gross in 1946.
This occurs in about 1 in every 4000 babies. If stillbirths are included, it can occur in up 1 in 2000 births. The hole can vary a lot in size from ½ to 1 inch in size up to complete absence of the diaphragm on one side. Babies who have a large hole in the diaphragm often have problems right after birth and many don’t survive. In general, survival is related to the how small the lung is on the side of the hernia and the opposite side. Most holes in the diaphragm (80%) are on the left side and most of these contain the small intestine, the spleen, stomach, and colon. Right-sided hernias usually contain part of the liver and intestine.
Several drugs can be used to try to help this situation. Some drugs (such as nitric oxide) can make the pressure in the lung arteries lower.
The baby may have a flat abdomen at birth and the heart is pushed to the side opposite from the diaphragmatic hernia. X rays of the baby may show some of the intestine in the chest cavity (Figure 2). These may show the heart shifted to the other side as well. There is another rare lung problem that can look like CDH (cystic adenomatoid malformation), but if there is a question, an upper GI series can show the difference.
Other problems happen in 15% to 25% of babies with CDH, the most common being heart defects. Problems such as holes in the heart (ventricular septal defect), small heart (hypoplastic heart) and others can be seen. Some babies also have chromosome problems, brain problems and others.
The operation may make the lungs worse because it can put The operation may make the lungs worse because it can put pressure on the good lung. This is usually a problem for just a short time. Before surgery, the baby will usually have a tube going into the stomach and catheters in an artery and vein. The infant will be on a mechanical ventilator and most hospitals will try to keep the amount of pressure on the lungs from the ventilator as low as possible. This is to avoid injuring the very small lungs.
Before surgery, the baby will have a tube in the stomach to keep air out of the intestines and to keep the intestines from pressing on the lungs. The amount of oxygen in the baby’s blood will be watched constantly, usually with a device (pulse oximeter) on the hand or foot. The baby may need medicines to keep the blood pressure normal. In some hospitals, the baby may have a different ventilator that works very fast to breathe for the infant (a high frequency ventilator).
If the baby does not keep the blood oxygen level high enough with these devices, heart lung bypass (ECMO) may be used for several days to weeks. This can only be done in very specialized centers and the infant may be transferred to a center that does ECMO.
The baby will usually have surgery after the situation is stable. It may be days or weeks before an operation is done. If the baby is on ECMO, the infant may have the surgery while on the ECMO machine. The operation may make the lungs worse initially. This is because the intestines are put back into the abdomen and cause pressure on the lungs from below.
After this is done, the edges of the diaphragm are found. If they can be sewn together, they will be (Figures 5, 6, 7). Sometimes, the hole is too large. If this is the case, an artificial patch (such as Gore-Tex) may be used to close the hole (Figure 8 and 9). If, after fixing the hole, there is not enough room in the abdomen for the intestines, another patch may be placed on the abdomen to hold the intestines until the swelling gets better.
The best time for the operation is still debated, but currently most surgeons wait until the baby does not need a lot of help from the ventilator. As mentioned, this can sometimes take days or weeks.
Although appealing, repair of the CDH while the baby is still in the uterus (fetal surgery) has not been shown to be of help. There is still experimental work being done in attempts to grow the lungs in utero, but only in select situations.
There are other experimental projects in babies with CDH including transplanting the lung and using a form of liquid in the lungs to help lung growth. Neither of these is widely available or of proven benefit.
Infants with CDH are usually in the hospital for weeks to a few months after the diaphragm is fixed. This mostly depends on how sick the baby is and how small the lungs are. After the baby goes home, they will be followed closely to make sure the hernia doesn’t recur. This can happen quite often, especially in babies with a patch repair of the diaphragm. This is because the baby will be growing, but the patch can’t, so the patch may slowly pull away.
About 2/3 of all babies with CDH who are sick at birth will live. Some of these babies can have other severe problems such as heart defects and many of those babies don’t live. About 80% of infants with CDH and no other problem will live if they are cared for in specialized centers. As mentioned above, these babies do need long-term follow up care as some of them will have other problems.
Other Diaphragm Problems
Foramen of Morgagni Hernia
Surgery can be done
through an incision in the upper abdomen or it can also be
done using a laparoscope through small incisions. The intestines
in the hernia are placed back in the abdomen and the diaphragm is then
Most patients do well after this operation.
Eventration of the Diaphragm
Babies with eventration are not usually as sick as the baby with a CDH. Many infants with congenital eventration have no symptoms, whereas most children with acquired eventration develop significant symptoms. Children with no symptoms can be watched (Figure 11). Those who have breathing difficulty or who cannot be weaned from a mechanical ventilator require an operation.
The operation to
fix an eventration can be done through an incision in the chest
or the abdomen. The diaphragm is tightened by removing
the very thin areas of the diaphragm and closing the strong areas
closer together. Pleats of diaphragm (placation) may be formed.
One must be careful to avoid injuring the nerve to the diaphragm.
This problem can occur after a fundoplication
as well. It is often noted on a routine chest x-ray. Surgery to
the amount of stomach that has slipped into the chest and whether
there are symptoms or not.
Adzick NS, Harrison MR, Glick PH, et al: Diaphragmatic
hernia in the fetus: Prenatal diagnosis and outcome in 94 cases.
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Boloker J, Borteman D, Wung JT, Stolar CJA:
Congenital diaphragmatic hernia in 120 infants treated consecutively
with permissive hypercapnia,
spontaneous respiration and elective repair. J Pediatr Surg 37:357,
Clark RH, Hardin
WD, Hirschl RB, et al: Current surgical management of congenital
diaphragmatic hernia: A report from the Congenital
Diaphragmatic Hernia Study group. J Pediatr Surg 33:1004, 1998.
MR, Keller RL, Hawgood SB, et al. A randomized trial of fetal
endoscopic tracheal occlusion for severe fetal congenital
Articles and graphics adapted from O'Neill: Principles of Pediatric Surgery. © 2003, Elsevier.