Diaphragmatic hernia repair - congenital

Definition

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.

Description

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.

Indications
When the abdominal organs pass into the chest cavity, the lung tissue on the affected side is compressed, fails to grow normally, and is unable to expand after birth. As the child begins to breathe, cry, and swallow, air enters the intestines that are protruding into the chest. The increasing size of the intestines puts pressure on the other side of the chest, the other lung, and the heart and can quickly cause a life-threatening situation.

The indications for a diaphragmatic hernia repair include the following:

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

Risks
Risks for any anesthesia include the following:

     
  • Reactions to medications  
  • Problems breathing

Risks for any surgery include the following:

     
  • Bleeding  
  • Infection

Other possible complications include the following:

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

Expectations after surgery

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.

Convalescence
Babies may require several weeks of hospitalization after surgery depending on how long breathing needs to be supported with a machine. Feeding is begun after the first bowel movement is passed. Feeding is usually done through a tube into the stomach or small intestines until the breathing tube is removed.

Johns Hopkins patient information

Last revised: December 2, 2012
by Arthur A. Poghosian, M.D.

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