Fetoscopic Balloon Dilation and Stent Placement of Congenital High Airway Obstruction Syndrome Leading to Successful Cesarean Delivery

Abstract

Introduction: Without fetal or perinatal intervention, congenital high airway obstruction syndrome (CHAOS) is a fatal anomaly. The ex utero intrapartum treatment (EXIT) procedure has been used to secure the fetal airway and minimize neonatal hypoxia, but is associated with increased maternal morbidity. Case Presentation: A 16-year-old woman (gravida 1, para 0) was referred to our hospital at 31 weeks gestation with fetal anomalies, including echogenic lungs, tracheobronchial dilation and flattened diaphragms. At 32 weeks, fetoscopic evaluation identified laryngeal stenosis, which was subsequently treated with balloon dilation and stent placement. The patient developed symptomatic and regular preterm contractions at post-operative day 7 with persistent sonographic signs of CHAOS, which prompted a repeat fetoscopy with confirmation of a patent fetal airway followed by Cesarean delivery under neuraxial anesthesia. Attempts to intubate through the tracheal stent were limited and resulted in removal of the stent. A neonatal airway was successfully established with rigid bronchoscopy. Direct laryngoscopy and bronchoscopy confirmed laryngeal stenosis with a small tracheoesophageal fistula immediately inferior to the laryngeal stenosis and significant tracheomalacia. A tracheostomy was then immediately performed for anticipated long term airway and pulmonary management. The procedures were well tolerated by both mom and baby. The baby demonstrated spontaneous healing of the tracheoesophageal fistula by day of life 7 with discharge home with ventilator support at three months of life. Conclusion: Use of repeated fetoscopy in order to relieve fetal upper airway obstruction offers the potential to minimize neonatal hypoxia, while concurrently decreasing maternal morbidity by avoiding an EXIT procedure. Use of the tracheal stent in CHAOS requires further investigation. The long-term reconstruction and respiratory support of children with CHAOS remain challenging

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

“…While the aforementioned case reports have described instances such as fetal intubation to avoid EXIT procedure in the setting of an unstable fetal airway or the ability to secure a definitive fetal airway at the time of fetal laryngoscopy and intervention for prenatally diagnosed CHAOS [5, 12, 19, 21], given the nature of this patient’s airway malformation, it was not felt that additional fetoscopic intervention would have yielded any benefit to secure a definitive airway and avert an EXIT procedure. This was confirmed at the time of near-term delivery, when an oral airway was unable to be secured by ENT services, and tracheostomy was ultimately performed.…”

“…Fetoscopic evaluation of the fetal airway with subsequent intervention has been described for a variety of indications including: (1) fetal intubation attempt prior to delivery for prenatally diagnosed airway obstruction in lieu of the more invasive outright EXIT procedure [5,12], (2) fetal endoscopic tracheal balloon occlusion in the setting of moderate or severe congenital diaphragmatic hernia [13][14][15], and (3) surgical recanalization or decom-pression of the fetal airway in the setting of CHAOS, tracheal cyst or bronchia atresia [16][17][18][19][20][21].…”