Successful Fetal Intervention for Congenital High Airway Obstruction Syndrome

Abstract

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.

A case of congenital high airway obstruction syndrome caused by complete tracheal obstruction with associated anomalies

“…It was possible to create a pharyngotracheal fi stula for the decompression of the lung. However, further studies are necessary to determine the long term effects and safety of fetoscopic tracheoscopy [2,10]. In the case presented, the fetus with CHAOS not only had severe hydrops, but also had associated anomalies.…”

Antenatal ultrasonographic diagnosis of congenital high airway obstruction syndrome - a chaotic state: a case report

“…8,9 Even with the presence of hydrops fetalis, successful fetal intervention by tracheostomy and EXIT procedure were recorded . 10,11 CHAOS is a rare and fatal cause of congenital airway obstruction if unrecognized during prenatal period. Antenatal sonographic imaging shows typical findings which can lead to a diagnosis.…”

Airway tissue engineering for congenital laryngotracheal disease

“…In cases of extensive tracheooesophageal connections or CDH, the opposite problem where babies are born with hypoplastic lungs may also occur because the fetus has been unable to generate intrapulmonary negative pressure in utero [10]. Fetal surgical techniques have gained acceptance in a variety of conditions including CDH [11,12], and fetal tracheostomy is gaining momentum as a viable treatment option to relieve tracheal obstruction in some fetal surgical centers [13].…”