Congenital high airway obstruction syndrome (CHAOS): virtual navigation in the fetal airways after intrauterine endoscopic treatment

ABSTRACT Background Congenital high airway obstruction syndrome (CHAOS) involves the partial or complete obstruction of the fetal upper airways, usually caused by atresia or stenosis of the larynx or trachea. The obstruction of bronchial tree leads to lung distension, diaphragmatic eversion, and cardiac dysfunction, which can result in fetal death. Case A primigravid 19-year-old was diagnosed with CHAOS at 193 weeks gestation. Virtual navigation using magnetic resonance imaging (MRI) data was used to visualize the fetal airways after intrauterine endoscopic laser decompression. A perforation in the fetal larynx/trachea was identified and the diagnosis was modified to tracheal stenosis. Caesarean delivery occurred at 315 weeks using an ex utero intrapartum treatment (EXIT) procedure. The neonatology team were unable to perform intubation, suggesting a final diagnosis of tracheal atresia. The male newborn weighed 1920 g and died one hour later. Conclusion 3D virtual bronchoscopy is a non-invasive approach to visualizing the fetal upper airways and can be used to diagnose and manage CHAOS.