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Key Points

  1. The 2 most critical elements of congenital diaphragmatic hernia (CDH) are pulmonary hypertension and hypoplasia.

  2. While a diagnosis of diaphragmatic hernia is theoretically possible from the 12th week on, in reality, the earliest CDH can be detected with current ultrasonographic equipment is 14 to 15 weeks, or late in the first and early in the second trimester.

  3. Left-sided CDH is found in 80% to 85% of cases; right-sided defects in 10% to 15% of patients in different series. Rarely are the defects bilateral.

  4. Most infants with a diaphragmatic hernia will present at birth or shortly after with varying degrees of respiratory distress.

  5. Antenatal intervention for severe CDH is an appropriate therapeutic option in very few patients.

  6. The first line of treatment of a newborn with suspected or documented diaphragmatic hernia is respiratory support.

  7. The key to a successful CDH repair rests in the security of the sutures and the freedom from tension on the repair.

  8. Innovative approaches to endoscopic and even laparoscopic efforts at repair now are considered routine for 25% to 45% of newborns with CDH.

  9. For infants in immediate distress in the delivery room or intensive care unit, extracorporeal membrane oxygenation (ECMO) may be lifesaving and is considered by many as mandatory for supporting infants with CDH and in managing high-risk deliveries of fetuses with known CDH.

  10. Major institutions in the United States have reported survival outcomes for patients with CDH that range from 70% to 85%.

  11. For patients with diaphragmatic herniation through the foramen of Morgagni, operative reduction and repair is generally easily accomplished through the abdomen using open or minimally invasive techniques.

  12. Traumatic hernias may be repaired through an abdominal approach during exploration following blunt or penetrating trauma. If recognition of the trauma has been delayed, a transthoracic incision or thoracoscopic approach might be preferable.

  13. Congenital or acquired abnormalities of the diaphragm and phrenic nerve can lead to unilateral or bilateral diaphragmatic eventration that can be repaired through abdominal and thoracic open approaches and minimally invasive surgery.


The 2 most critical elements of congenital diaphragmatic hernia (CDH) are pulmonary hypertension and hypoplasia. Pulmonary hypertension was first demonstrated in CDH patients in 1971 by Rowe and Uribe. The associated morphologic finding of increased arteriolar muscularization is the foundation for the persistent pulmonary hypertension of the newborn (PPHN), leading to persistent fetal circulation (PFC). Morphometric examinations of the lungs of infants who have died with CDH, as well as of experimental animals with CDH, demonstrate uniform loss of pulmonary mass and decreased bronchial branching as well as alveolar:arteriolar ratios. Pulmonary hypoplasia resulting from the arrest of alveolar development at the midcanalicular stage is a central factor in mortality, long-term morbidity, and restrictions on the quality of outcomes with CDH.

The present and future medical and surgical management of CDH has been one of the central focal points of pediatric surgery for the past 25 years. Several ...

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