What is congenital diaphragmatic hernia?

Diaphragmatic hernia is a condition that can be discovered before or after birth. Learn about diaphragmatic hernia symptoms and how babies are treated for the condition.

Diaphragmatic hernia is a hole in the diaphragm caused by a birth defect. The diaphragm is the large muscle that separates the chest from the abdomen. In a diaphragmatic hernia, organs such as intestines, spleen, stomach and liver move through the hole in the diaphragm into a baby’s chest. A hernia is a condition in which part of an organ is displaced and protrudes through the wall of the cavity containing it.

The organs pushing through the hole in the diaphragm is defined as a hernia. This type of hernia can prevent a baby’s lungs from developing as they should, a condition known as pulmonary hypoplasia.

Children can become more at risk for other problems due to pulmonary hypoplasia, including:

  • Asthma
  • Developmental delays
  • High blood pressure in the pulmonary circulation (pulmonary hypertension)
  • Feeding disorders
  • Gastrointestinal reflux
  • Reduced blood flow to the lungs

Diaphragmatic hernia types

There are two types of diaphragmatic hernia, including:

  • Bochdalek hernia. A Bochdalek hernia affects the side and back of the diaphragm as the diaphragm does not develop as it should. The intestine may become trapped in the chest cavity, and the stomach, liver, spleen, and/or intestines usually move up into the chest cavity with this type of hernia. Bochdalek hernia is the most common type of diaphragmatic hernia.
  • Morgagni hernia. A Morgagni hernia usually affects the front part of the diaphragm. In this type, the tendon that should develop in the middle of the diaphragm doesn’t form as it should. The liver and/or intestines move up into the chest cavity with this type of hernia. This kind is much rarer.

How is a diaphragmatic hernia diagnosed?

A diaphragmatic hernia can be diagnosed in the womb or after birth. If an OB/GYN suspects the condition, tests can confirm a diagnosis. A fetal echocardiogram, magnetic resonance imaging (MRI) scan and more tests may be ordered. A baby with diaphragmatic hernia will be closely monitored in the womb.

A child can be diagnosed immediately after birth, with many of the same tests ordered, if he or she shows symptoms of the condition.

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What are diaphragmatic hernia symptoms?

Symptoms of a diaphragmatic hernia are often noticeable soon after a baby is born. Symptoms may vary based on how each child experiences them. A baby with a Morgagni hernia may or may not show any symptoms until later in life. Symptoms of a Bochdalek diaphragmatic hernia may include:

  • Abdomen appears caved-in
  • Blue color of the skin (cyanosis)
  • Fast breathing
  • Fast heart rate
  • One side of the chest is larger than the other
  • Trouble breathing

How is a diaphragmatic hernia treated?

After the baby is born, he or she will begin treatment for diaphragmatic hernia. The following may happen:

  • The baby will be moved to the neonatal intensive care unit (NICU).
  • The baby may need to be put on a breathing machine (mechanical ventilator) and possibly placed on extracorporeal membrane oxygenation (ECMO), if needed. This will help stabilize the baby so he or she can be ready for surgery. ECMO is a special type of heart lung bypass for infants and children with reversible causes of lung or heart disease. Depending on the child’s progress, surgery can happen as early as the second or third day after birth. If the baby is on ECMO, the surgery can be done on ECMO although there is an increased risk of bleeding while on ECMO and the surgeon will want to come off ECMO soon after repair. Some babies who are premature may be too small to qualify for ECMO.
  • When it’s time for surgery, the baby will get general anesthesia. The baby is monitored by a pediatric anesthesiologist during the entire procedure.
  • Surgeons repair the diaphragmatic hernia: An incision is made just below the rib cage and the surgeon guides the organs back down into the abdomen. The hole in the diaphragm is sewn closed, or if the diaphragm is not present, an artificial diaphragm is constructed.
  • The newly created space in the chest will allow the lungs to continue growing with more needed air sacs (alveoli).
  • A child’s recovery and length of hospital stay can vary depending on the hernia type and the child’s response to treatment. The baby usually needs breathing support with a ventilator for at least a short time after surgery.
  • A child may need oxygen and medications to help with breathing for weeks, months or years after being removed from the ventilator depending on the degree of pulmonary hypoplasia.