Topic summary

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a rare birth defect that occurs when incomplete formation of the diaphragm allows abdominal organs to protrude into the chest. This herniation of abdominal organs compresses developing lung tissue and impairs lung development, typically causing respiratory distress shortly after birth. Most defects occur in the posterolateral portion of the diaphragm and are known as Bochdalek hernias. The cause of the defect is generally unknown. The severity of CDH ranges from mild to life-threatening and is closely related to the degree of pulmonary hypoplasia and pulmonary hypertension. Treatment of CDH begins with cardiorespiratory stabilization, followed by surgical repair of the diaphragmatic defect. CDH occurs at a frequency of 2-3 cases per 10,000 births.