2019 Volume 55 Issue 4 Pages 815-822
Purpose: We reviewed our experience in treating patients with congenital laryngeal atresia to evaluate their clinical outcomes and the problems encountered when treating them.
Methods: This retrospective study included nine patients with congenital laryngeal atresia, who were managed at our institutions between 1982 and 2017.
Results: Seven patients developed fetal ascites. A prenatal diagnosis of congenital high airway obstruction syndrome (CHAOS) was made at a median gestational age of 21 weeks in five patients. EXIT procedures were performed in four of five patients with CHAOS. In the other two patients who were dichorionic diamniotic twins, intrauterine fetal death occurred in one of the twins owing to fetal heart failure. The airway was successfully accessed in the four patients in whom tracheostomy was performed under an EXIT procedure. Although tracheostomy was performed immediately after birth in four patients in whom CHAOS was not prenatally diagnosed, the patients died of hypoxemia, except for one patient with tracheoesophageal fistula and esophageal atresia. The median gestational age of the neonates was 37 weeks, and the median body weight was 2,015 g. Congenital anomalies were confirmed in five patients. Five patients survived during the neonatal period, whereas two patients died owing to complications of the underlying diseases. Long-term survival was only achieved in three patients.
Conclusion: We concluded that the EXIT procedure was effective for the airway management of fetuses with congenital laryngeal atresia and that it improved the outcomes of such patients.
