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.
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.
Authors: Kate G Ackerman; Jianlong Wang; Liqing Luo; Yuko Fujiwara; Stuart H Orkin; David R Beier Journal: Am J Respir Cell Mol Biol Date: 2006-12-01 Impact factor: 6.914
Authors: Oluyinka O Olutoye Ii; Walker D Short; Jamie Gilley; J D Hammond Ii; Michael A Belfort; Timothy C Lee; Alice King; Jimmy Espinoza; Luc Joyeux; Krithika Lingappan; Jason P Gleghorn; Sundeep G Keswani Journal: Front Pediatr Date: 2022-07-05 Impact factor: 3.569
Authors: Kate G Ackerman; Bruce J Herron; Sara O Vargas; Hailu Huang; Sergei G Tevosian; Lazaros Kochilas; Cherie Rao; Barbara R Pober; Randal P Babiuk; Jonathan A Epstein; John J Greer; David R Beier Journal: PLoS Genet Date: 2005-06-17 Impact factor: 5.917