Diaphragmatic Hernia (Congenital Diaphragmatic Hernia) (CDH)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Etiology/Pathophysiology:
Due to a congenital failure of the diaphragm to close correctly. The most common diaphragmatic hernia in the newborn is Bochdalek which is posterolateral in location and five times more common on the left than on the right due to the presence of the liver on the right. The degree of ipsilateral pulmonary hypoplasia is based on the size of the hernia and what developmental stage it occurred at in utero and interrupted pulmonary growth. Cases with large hernias can also have contralateral pulmonary hypoplasia. Persistent fetal circulation and pulmonary hypertension can be complications of this arrested pulmonary development.

Pathology:
Distinct abnormal openings in the diaphragm are seen at surgery.

Imaging Findings:
Herniated bowel (stomach, small bowel, colon) and solid viscera (liver, omentum, spleen) are seen in the ipsilateral hemithorax causing mediastinal shift. If seen immediately after birth, the hemithorax may be opaque, until the bowel fills with gas . Confirmation that the cystic lucencies in the chest represent air in bowel can be made via insertion of a nasogastric tube into the stomach to define its position, or via an upper GI exam . The Bochdalek hernia is five times more common on the left than on the right due to the presence of the liver on the right . Once repaired, the ipsilateral and contralateral pulmonary hypoplasia is well seen, as the lungs often are too small to expand to completely fill their hemithoraces.

DDX:

• Congenital Pulmonary Airway Malformation - Usually has multiple cysts, unlike congenital lobar emphysema which has only one cyst. The abdominal gas pattern is normal.
• Congenital lobar emphysema - bowel gas is in a normal position.

References

• Correlapedia
• Correlapedia
• Thoracopedia

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