Airway anomalies in patients with congenital diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernias (CDH) sometimes are associated with 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 1999. 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 defect of the right upper lobe bronchus, 2 had a tracheal bronchus, and 3 had a trifurcated trachea. Bronchial hypoplasia on the affected side was identified in 15 patients and was seen in all patients with anatomic anomalies of the tracheobronchial tree except the 2 with tracheal bronchus. After excluding 5 patients with severe associated anomalies, 6 of 14 patients with an abnormal 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 patients with CDH, respectively. CDH patients who exhibited these abnormalities showed a poor outcome.