Airway anomalies in patients with congenital diaphragmatic hernia

Purpose: Congenital diaphragmatic hernias (CDH) sometimes are associated wi th airway anomalies such as congenital stenosis, abnormal branching of the bronchi, and pulmonary hypoplasia. The incidence of these associated airway anomalies has not been reported previously. Methods: Bronchoscopy was performed in all neonates with CDH from 1987 to 1 999. In addition to anatomic anomalies, bronchial hypoplasia was defined as narrowing and shortening of the bronchi at bronchoscopy. Results: Anatomic anomalies were identified in 7 of 39 patients with CDH: 1 had congenital tracheal stenosis with pulmonary artery sling, 1 had a defe ct of the right upper robe bronchus, 2 had a tracheal bronchus, and 3 had a trifurcated trachea. Bronchial hypoplasia on the affected side was identif ied in 15 patients and was seen in all patients with anatomic anomalies of the tracheobronchial tree except the 2 with tracheal bronchus. After exclud ing 5 patients with severe associated anomalies, 6 of 14 patients with an a bnormal tracheobronchial tree died, whereas 1 of 20 patients without airway abnormalities died. Conclusions: Anatomic anomalies of the tracheobronchial tree and bronchial hypoplasia on the affected side were identified in 17.9% and 38.4% of patie nts with CDH, respectively. CDH patients who exhibited these abnormalities showed a poor outcome. Copyright (C) 2000 by W.B. Saunders Company.