In utero Treatment of Congenital High Airway Obstruction Syndrome via Fetal Laryngoscopy and EXIT Procedure

• Published in: Fetal diagnosis and therapy Vol. 49; no. 9-10; pp. 385 - 393
• Main Authors: Sabra, Rana, Gheorghe, Ciprian P., Monson, Martha A., Masri, Jinnen, Chmait, Ramen H.
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland 01.02.2023
• Subjects: Airway Obstruction - diagnostic imaging; Airway Obstruction - surgery; Ex utero Intrapartum Treatment Procedures; Female; Fetal Therapy; Humans; Hydrops Fetalis - surgery; Infant, Newborn; Laryngoscopy - adverse effects; Pregnancy; Trachea - surgery; Congenital high airway obstruction syndrome; Fetoscopy; Ex-utero intrapartum treatment procedure; Laser
• ISSN: 1015-3837; 1421-9964
• DOI: 10.1159/000526798

Introduction: Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can progress to fetal hydrops and demise in utero or at birth unless interventions are undertaken to alleviate the tracheal obstruction. While the ex-utero intrapartum treatment (EXIT) procedure for airway stabilization is technically feasible, abnormal pulmonary development as a result of the antenatal obstructive process may result in severe postnatal respiratory complications. Case Presentation: We describe a case of CHAOS with secondary hydrops treated in utero at 24 0/7 weeks’ gestation by fetoscopic tracheal decompression via laser perforation of the airway obstruction. Interval imaging after the fetoscopic operation demonstrated resolution of the fetal hydrops. Tracheostomy for airway stabilization was performed at the time of the EXIT procedure near term (36 0/7 weeks). The patient underwent tracheal reconstruction and decannulation at 3 years of life. Discussion/Conclusion: The primary goal of fetoscopic airway evaluation and intervention is not necessarily to perform definitive stabilization of the airway but rather to achieve sufficient decompression of the trachea to reverse fetal hydrops and salvage pulmonary development. In utero fetoscopic treatment may allow for prolongation of the pregnancy with delivery at or near term via EXIT procedure for definitive neonatal airway stabilization.