Diaphragmatic hernia repair - congenital

  • Definition
    • Diaphragmatic hernia repair is surgery to correct an opening or space in a baby's diaphragm. This opening is called a hernia. It is a rare type of birth defect. (Congenital means the problem is present at birth.)

  • Description
    • Before this surgery, nearly all infants need a breathing device to improve their oxygen levels.

      The surgery is done while your child is under general anesthesia (asleep and not able to feel pain). The surgeon makes a cut (incision) in the belly under the upper ribs. This allows the organs in the area to be reached. The surgeon gently pulls these organs down into place through the opening in the diaphragm and into the abdominal cavity.

      The surgeon repairs the hole in the diaphragm. If the hole is small, it may be repaired with stitches. In most cases, a piece of plastic patch is used to cover the hole.

  • Why the Procedure Is Performed
    • The diaphragm is a muscle. It is important for breathing. It separates the chest cavity (where the heart and lungs are) from the belly area.

      In a child born with a diaphragmatic hernia, the diaphragm muscle is not completely formed. So, organs from the belly (stomach, spleen, liver, and intestines) may go up into the chest cavity where the lungs are. This prevents the lung(s) from growing normally. The lungs stay too small for babies to breathe on their own when they are born.

      A diaphragmatic hernia can be life-threatening. Surgery to repair it must be done in the first few days or weeks of a child's life.

  • Risks
  • Before the Procedure
    • Infants with a diaphragmatic hernia are admitted to a neonatal intensive care unit (NICU). It may be days or weeks before the baby is stable enough for surgery.

      • In the NICU, your baby will probably need a breathing machine (mechanical ventilator) before the surgery. This helps the baby breathe.
      • If your child is very sick, a heart-lung bypass machine (ECMO) may be needed to do the work of the heart and lungs.
      • Before surgery, your baby will have x-rays and regular blood tests to see how well the lungs are working. A light sensor (called a pulse oximeter) is taped to baby's skin to monitor the oxygen level in the blood.
      • Your baby may be given medicines to control blood pressure and keep him or her comfortable.

      Your baby will have tubes placed:

      • From the mouth or nose to the stomach to keep air out of the stomach
      • In an artery to monitor blood pressure
      • In a vein to deliver nutrients and medicines
  • After the Procedure
    • Your baby will be on a breathing machine after the surgery and will stay in the hospital for several weeks. Once the baby is taken off the breathing machine, they may still need oxygen and some medicine for a while.

      Feedings will start after your baby's bowels start working. Feedings are usually given through a small, soft feeding tube from the mouth or nose into the stomach or small intestines until your baby can take all the milk they need by mouth.

      Most infants with diaphragmatic hernias have reflux when they eat. This means the food or acid in their stomach moves up into their esophagus, the tube that leads from the throat to the stomach. This can be uncomfortable for your baby. It also leads to frequent spitting up, vomiting, and pneumonia, which makes feedings more difficult once your baby is taking food by mouth.

      The nurses and feeding specialists will teach you ways to hold and feed your baby to prevent reflux. Some babies need to be on a feeding tube for a long time to get enough food to grow.

  • Outlook (Prognosis)
    • The outcome of this surgery depends on how well your baby's lungs have developed. Some babies have other medical problems, including problems with the brain, muscles, and joints, which may slow recovery.

      Usually, the outlook is good for infants who have well-developed lung tissue. Most babies who are born with a diaphragmatic hernia are very ill and will stay in the hospital for a long time. With advances in medicine, the outlook for these infants is gradually improving.

      All babies who have had diaphragmatic hernia repairs will need to be watched closely to make sure the hole in their diaphragm does not open up again as they grow.

      Babies who had a large opening or defect in the diaphragm, or who had more problems with their lungs after birth, may have lung disease after they leave the hospital. They may need oxygen, medicines, and a feeding tube for months or years.

      Some babies will have problems crawling, walking, talking, and eating. They will need to see physical or occupational therapists to help them develop muscles and strength.

  • References
    • Fauza DO, Wilson JM. Congenital diaphragmatic hernia. In: Selke FW, del Nido P, Swanson SJ. Sabiston and Spencer's Surgery of the Chest. 8th ed. Philadelphia, PA: Elsevier Saunders; 2010:chap 33.