Affiliation:
1. Division of Pulmonary Medicine Boston Children's Hospital Boston Massachusetts USA
2. Harvard Medical School Boston Massachusetts USA
3. Department of Surgery Children's Hospital of Philadelphia Philadelphia Pennsylvania USA
4. Department of Surgery Johns Hopkins All Children's Hospital St. Petersburg Florida USA
5. Department of Critical Care Medicine Boston Children's Hospital Boston Massachusetts USA
6. Division of Pulmonary Medicine Hassenfeld Children's Hospital at NYU Langone Health New York New York USA
7. Department of Surgery Boston Children's Hospital Boston Massachusetts USA
Abstract
AbstractBackgroundTracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular‐related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery.Materials and MethodsThe study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre‐ and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t‐test for continuous data.ResultsStatistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre‐ and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms.ConclusionAirway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long‐term studies are needed to confirm this benefit.