A congenital (present from birth) diaphragmatic hernia is caused by a defect in which the diaphragm, the large dome-shaped muscle that separates the chest cavity from the abdomen, fails to completely develop. The condition requires immediate surgery to repair the damage caused by the defect.

The incomplete diaphragm has a large hole through which the abdominal organs can pass (herniate) into the chest cavity. Most commonly the hole occurs on the left side (almost 90% of cases). This compresses the fetal lung nearest the hernia, preventing full development, and thus interferes with breathing after the infant is born.

The incidence of congenital diaphragmatic hernia is approximately 1 in 2,000 births, and boys are more commonly affected.

While the child is under general anesthesia, an incision is made in the upper abdomen, under the ribs. The abdominal organs are gently pulled down through the opening in the diaphragm and positioned into the abdominal cavity.

The hole in the diaphragm is repaired and the incision is stitched closed. If a large defect is present, a plastic patch is used to cover the defect in the diaphragm. A tube is placed in the chest to allow air, blood, and fluid to drain so the affected lung can re-expand. This tube remains in place after surgery for a few days.

• Chest X-rays showing diaphragmatic hernia
• Severe breathing difficulty (respiratory distress) shortly after birth
• Prenatal ultrasound often identifies a diaphragmatic hernia

• Reactions to medications
• Problems breathing

• Bleeding
• Infection

• Collapsed lung (pneumothorax)
• Failure of the affected lung to expand or mature

Diaphragmatic hernia is a life-threatening condition that requires surgery as soon as symptoms develop, usually in the first 24 hours of life. The outcome depends on the lung development on the affected side.

Most babies require ventilator support (the use of a machine to help them breathe) after surgery. Generally the prognosis is very good for infants with adequate lung tissue.