Percutaneous Fetoscopic Laser Decompression of Congenital High Airway Obstruction Syndrome (CHAOS) from Laryngeal Atresia via a Single Trocar – Current Technical Constraints and Potential Solutions for Future Interventions

Abstract

Objective: To alleviate congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia by percutaneous minimally-invasive fetoscopic tracheal decompression using laser. Methods: The procedure was performed via one trocar under general maternofetal anesthesia in a human fetus with CHAOS from laryngeal atresia at 21+6 weeks of gestation. Results: Normalization of the lung-heart size relationship was observed within days after the procedure. The fetus was delivered by ex utero intrapartum treatment (EXIT) in order to perform a tracheotomy at 31+1 weeks of gestation and survived hospital treatment to discharge. Conclusions: Percutaneous minimally-invasive fetoscopic decompression of the fetal trachea via a single trocar is feasible in human fetuses with CHAOS from laryngeal atresia.

Evaluation of Fetal Vocal Cords to Select Candidates for Successful Fetoscopic Treatment of Congenital High Airway Obstruction Syndrome: Preliminary Case Series

“…Only six attempts of fetal therapy for CHAOS have been reported [5,6,7,8,14]. In the first case, Ward et al [5] attempted an open surgery fetal tracheostomy at 18 weeks' gestation, but there was a fetal death a few hours later.…”

“…Unfortunately, diaphragmatic function remained impaired so that tracheotomy and ventilatory support were continued beyond discharge from hospital at 4 months of age. The infant died at home 2 weeks later [8]. Finally, Saadai et al [14] reported 2 cases which underwent fetal bronchoscopy with an attempted wire tracheoplasty, i.e.…”

“…In the surviving cases, the quality of lung tissue is poor and rarely allows normal respiratory function, possibly due to dysplastic features resulting from long-term exposure to high airway pressure. The long-term evolution of these children is also associated with exceedingly high morbidity and poor quality of life [4,7,8]. According to the small available evidence, if CHAOS is caused by isolated subglottic membrane, fetoscopy can be a short single-port curative procedure, which permits normalization of lung development.…”

“…In a recent report, Roybal et al [4] aimed to identify prenatal characteristics predictive of a milder postnatal course.The absence of hydrops was associated with less adverse outcome, although in general all survivors suffered severe morbidity, with breathing, feeding and speech limitations [4]. Given the poor reported outcome, several attempts to permeate the airways by means of open surgery or fetoscopy have been reported [5,6,7,8]. Initial attempts [5,6,7] were performed in cases with complex forms of laryngeal atresia and either died during the procedure or suffered severe sequelae.…”

“…Initial attempts [5,6,7] were performed in cases with complex forms of laryngeal atresia and either died during the procedure or suffered severe sequelae. In the most recently reported case, Kohl et al [8] reported a purely fetoscopic approach to achieve tracheal decompression in a fetus with apparently isolated tracheal obstruction, although the infant eventually died.…”