Successful ex utero intrapartum treatment (EXIT) procedure for congenital high airway obstruction syndrome (CHAOS) owing to laryngeal atresia

Abstract

Congenital high airway obstruction syndrome (CHAOS) caused by laryngeal atresia was diagnosed by prenatal ultrasound in a male fetus at 18-weeks-gestation. Findings included enlarged lungs, inverted diaphragms, dilated trachea distal to the obstruction, and ascites. At 35 weeks' gestation, a planned ex utero intrapartum treatment (EXIT) procedure was performed, allowing bronchoscopic evaluation of the airway and placement of a tracheostomy. The postnatal course was marked by gradual recovery of diaphragmatic function with corresponding weaning from conventional ventilation to positive airway pressure. The sustained improvement in diaphragmatic and pulmonary function after the EXIT procedure for laryngeal atresia is one of the first reported for this entity and provides encouragement for future attempts.