AET-31
Management of complete tracheal rings in a patient with tetralogy of Fallot
Lee H, Nelson J, Deutsch N
Children's National Medical Center, Washington, DC, United states
Introduction
Congenital tracheal anomalies usually present as respiratory distress, stridor, or respiratory tract infections in the first few days of life but can also be asymptomatic. If mechanical ventilation is not possible, ECMO or stenting may be temporizing measures until surgical intervention is performed.
Case Report
A 3 day old female, born full term, was postnatally found to have tetralogy of Fallot (ToF) with critical pulmonary stenosis and pulmonary valve obstruction. She was prostaglandin-dependent, stable on room air, and tolerating feeds in the CICU while awaiting repair. On day 3 of life, she was found unresponsive with desaturations to the 30s. The CICU team bag mask ventilated and attempted multiple intubations with CMAC 1 blade. Each attempt yielded a grade I view, but they were unable to intubate. The patient was easy to bag mask ventilate between attempts. Anesthesiology was called for assistance, but we were unable to advance a 2.5 or 2.0 endotracheal tube due to an obstruction immediately distal to the vocal cords. Bedside echo confirmed adequate pulmonary blood flow. The patient was transported to the operating room for direct laryngoscopy and bronchoscopy. Upon insertion of the rigid bronchoscope, complete tracheal rings were found immediately distal to the vocal cords to the carina. The surgeon was unable to insert a 2.0 endotracheal tube. Tracheostomy was not an option due to severe long-segment stenosis. The airway was temporized with an LMA as the cardiac surgeons were called to cannulate the patient for VA ECMO. The right internal jugular vein and common carotid artery were cannulated with adequate flows.
Discussion
Our patient was born with not only life threatening tracheal stenosis, but also a severe cyanotic congenital heart defect. Oxygenation and ventilation were particularly concerning due to the risk of tet spells with hypoxia, hypercarbia, and acidosis. In addition to the risks of ECMO cannulation, which include infection, bleeding, and neurologic injury, there were other factors to consider for this patient. First, ECMO would require closure of the PDA to decrease overflow through the PDA to her lungs. The arterial cannula was inserted into the right common carotid artery and advanced to the descending aorta to decrease blood flow through the PDA. The prostaglandin was turned off after cannulation. Secondly, the patient would require cardiopulmonary bypass for the ToF repair and slide tracheoplasty. Surgeons note improved surgical exposure and more precise repair without an endotracheal tube obstructing the field. Remaining on ECMO postoperatively would also be preferred in this patient as unintentional extubation could result in difficult reintubation of the newly reconstructed trachea.
Conclusion
ECMO can be used as a bridge to slide tracheoplasty or tracheal reconstruction in patients who cannot be conventionally ventilated due to severe or long-segment stenosis.
References
1. Kunisaki SM et al. Extracorporeal membrane oxygenation as a bridge to definitive tracheal reconstruction in neonates. Journal of Pediatric Surgery. 43(5):800-804.
2. Schweiger C et al. Tracheal and bronchial stenoses and other obstructive conditions. Journal of Thoracic Disease. 2016;8(11):3369-3378.
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