Congenital diaphragmatic hernia The pathogenesis of this disorder is caused by the incomplete formation of the diaphragm in the fetus, resulting in a migration of the abdominal viscera into the chest during development. If the defect is large and the abdominal viscera have caused long-standing compression of the developing lungs, pulmonary hypoplasia may develop.
The diagnosis of diaphragmatic hernia is established frequently by prenatal ultrasound, which allows the management to be transferred to a perinatal referral center where pediatric surgery and appropriate medical support are available, including extracorporeal bypass.
In the delivery room, the infant often presents with respiratory distress. Physical signs may include a scaphoid abdomen and a shift in heart sounds to the right hemithorax.
Respiratory distress in the delivery room may be caused by either a pulmonary hypoplasia or may be secondary to an expansion of the bowel caused by swallowed air. The expansion of the bowel results in compression of the lung. Delivery room management includes immediate intubation and passage of a large catheter for gastric decompression. Intubation prevents distention of the stomach and bowel contents because of crying or bag-valve-mask ventilation. The gastric decompression should be achieved with a Replogle or Salem pump suction catheter connected to a low continuous drain.
Constant maintenance of the gastric suction during the preoperative and immediate postoperative period is essential.
New modes of ventilation such as high frequency oscillatory ventilation has decreased the use of extracorporeal membrane oxygenation (ECMO) in this population. However, the survival rate for infants with this anomaly has not changed over the past decade.
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