IS Case 307: Congenital diaphragmatic hernia
Imaging Sciences URMC 2009
Publication Date: 2009-09-11
Patient is a 2-day-old infant with cyanosis at birth.
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.
- Areechon W, Reid L. Hypoplasia of the lung with congenital diaphragmatic hernia. Br Med J 1963 Jan 26;1(5325): 230-233. PMID 14013675
- Donnelly LF. Pediatric Imaging: The Fundamentals. Saunders, 2009: 37-38.