IS Case 307: Congenital diaphragmatic hernia

Benita Tamrazi, MD

Imaging Sciences URMC 2009
Publication Date: 2009-09-11

History

Patient is a 2-day-old infant with cyanosis at birth.

Findings

Loops of bowel occupy the left hemithorax with associated cardiothymic shift to the right.

Discussion

Congenital diaphragmatic hernia (CDH) occurs in 1 of every 2,000-3,000 live births and accounts for 8% of all major congenital anomalies. There are three basic types which include the posterolateral Bochdalek hernia, the anterior Morgagni hernia, and the hiatus hernia. The left-sided Bochdalek occurs in 85% of cases. In the case of left-sided hernias, both the small and large bowel and intra-abdominal solid organs enter the thoracic cavity. Due to this, there is a variable degree of pulmonary hypoplasia with decreased pulmonary vasculature and dysfunction of the surfactant system.

The ideal time to repair a CDH is unknown. It has been suggested that repair 24 hours after stabilization is ideal, but delays of up to 7-10 days are typically well tolerated. There is high risk for significant long term morbidity including chronic lung disease, growth failure, and gastroesophageal reflux.

References

  1. Areechon W, Reid L. Hypoplasia of the lung with congenital diaphragmatic hernia. Br Med J 1963 Jan 26;1(5325): 230-233. PMID 14013675
  2. Donnelly LF. Pediatric Imaging: The Fundamentals. Saunders, 2009: 37-38.

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