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Diaphragmatic hernia repair is a surgical procedure to correct a congenital, or present at birth, defect (diaphragmatic hernia) in the diaphragm.
The diaphragm is the muscle that separates the chest cavity, which includes your heart and lungs, from the abdominal cavity. It is formed in the eighth week of gestation. Sometimes it does not form correctly, leaving a hole in the muscle. Most of the time, this hole occurs on the left side. This malformation causes the kidneys, spleen, liver, stomach, and intestines to rise into the chest, stunting the growth of the lungs (pulmonary hypoplasia).
Before a baby is born, the lungs are not needed to breathe, but underdeveloped lungs are life-threatening for a newborn. Surgical repair is almost always required.
Sometimes, routine prenatal imaging, such as an ultrasound or amniocentesis, will discover diaphragmatic hernia before a child is born. An ultrasound (or sonogram) is a test that uses sound waves to show a picture of the baby in the womb. Amniocentesis is used in conjunction with an ultrasound, and involves taking a sample of amniotic fluid to check for genetic disorders. Amniotic fluid is the liquid that surrounds the baby in the womb.
If diaphragmatic hernia is discovered, arrangements should be made for delivery in a perinatal center.
Before the repair can take place, your baby must be strong enough to withstand surgery. In some cases, the baby will be placed in a neonatal intensive care unit for a few weeks before surgery. A neonatal intensive care unit is a special wing of a hospital where babies receive around-the-clock monitoring and medical care. This unit is also housed with special equipment to help babies develop properly. Your child might need to be placed on a ventilator machine to help with breathing. In severe cases, a heart-lung bypass machine may be required.
Surgery is performed under general anesthesia, which means your child is put to sleep and feels no pain, and generally takes place within the first days or weeks of life.
During the procedure, the surgeon makes an incision and carefully returns the abdominal organs to their appropriate positions. The hole is then patched with a piece of mesh or repaired with stitches.
As with any surgery, there is a risk of bleeding and infection. Other risks for this procedure are:
Talk with your doctor about any concerns you may have.
Breathing assistance may still be needed for a few days after surgery. Usually, a chest tube is needed as well. This is to drain excess fluids and air from the chest.
After the surgery, feedings will most likely need to be given with a feeding tube to make sure your baby receives adequate nutrients. Babies who have had diaphragmatic hernias have a greater chance of having reflux (where acid or food rises up into the esophagus) when eating. This can cause more spitting up and vomiting. Ask your healthcare professionals about the best ways to position your baby for feedings.
Your baby may need to remain in the hospital for a few weeks before going home.
The lungs will now have the space to grow and mature.
The long-term outlook for this condition varies greatly from patient to patient. A lot depends on the extent of prenatal lung development.
Sometimes, prenatal imaging will discover diaphragmatic hernia before a child is born. In these cases, arrangements can be made to deliver in a perinatal center, which is specially equipped for special-needs or at-risk pregnancies. Such preparation can increase the infant’s odds of a favorable outcome.
Often, infants with congenital diaphragmatic hernia are very weak and must remain in the hospital for a considerable length of time.
Some children have other problems with their lungs or additional health issues that make recovery more difficult. Some will face long-term health issues. Developmental delays are possible, but physical and occupational therapy can improve muscle tone and strength.
Many infants who undergo diaphragmatic hernia repair go on to live normal lives. However, it is possible for the hole to open up again as your child grows.
In all cases, monitoring and follow-up medical care is necessary.
Medical advances, including less invasive surgical methods, are improving the outlook for babies born with this congenital defect.
Written by: Ann Pietrangelo
Medically reviewed : George Krucik, MD
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