Successful fetal intervention for congenital high airway obstruction syndrome.

INTRODUCTION: Complete congenital high airway obstruction syndrome (CHAOS) usually leads to stillbirth or death within minutes of delivery. We describe the management and long-term follow-up of a baby with a prenatally diagnosed airway obstruction.

CASE: Because of progressive hydrops fetalis, massive ascites and everted diaphragms due to CHAOS in a fetus at 24 weeks of gestation, a fetal tracheostomy was performed. Persistent fetal bradycardia at the time of the fetal procedure led to delivery of the infant. Adequate oxygenation and ventilation were attained using relatively low oxygen concentrations and low ventilatory pressures. Plain radiographs demonstrated that the lungs were large and the ribs thin. The infant has a permanent tracheostomy, required continuous assisted ventilation for several months and assisted ventilation at night at home for 3 years. At 4 years of age, he has no speech, all feedings are by gastrostomy, and developmentally he is mildly to moderately delayed.

CONCLUSION: Laryngeal atresia produced over-distended lungs and hydrops. Precise prenatal imaging and fetal surgical strategies may allow the survival of affected fetuses.