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Anesthetic management of an ex utero intrapartum treatment procedure for congenital high airway obstruction syndrome with failed fetal airway

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Sir,Congenital high airway obstruction syndrome (CHAOS) is a rare and potentially fatal fetal condition resulting from complete or near-complete obstruction of the upper airway. The condition is associated with severe neonatal respiratory compromise immediately after birth, making prompt airway establishment essential for survival. The ex utero intrapartum treatment (EXIT) procedure allows continuation of uteroplacental circulation while the fetal airway is secured and has emerged as a life-saving intervention in such scenarios. We report the anesthetic management of an EXIT procedure performed for a fetus diagnosed antenatally with CHAOS, highlighting the challenges encountered during failed fetal airway access and the critical role of anesthesia in maintaining placental perfusion. A 30-year-old gravida 2, para 1 woman at 36 weeks of gestation was referred to our tertiary care center following antenatal imaging suggestive of CHAOS. After multidisciplinary consultation involving obstetricians, anesthesiologists, pediatric surgeons, neonatologists, and radiologists, an elective EXIT procedure was planned. In the operating theater, invasive arterial blood pressure monitoring was instituted before induction of anesthesia. General anesthesia was administered to facilitate profound uterine relaxation and ensure optimal surgical exposure. Anesthesia was maintained with high concentrations of volatile agents. Maternal hemodynamic were supported using a phenylephrine infusion to preserve uteroplacental perfusion. Controlled uterine relaxation during the EXIT phase was achieved with a titrated nitroglycerine infusion. Following uterine incision, partial delivery of the fetal head and upper torso was performed while maintaining intact placental circulation. Multiple attempts at fetal airway establishment, including direct laryngoscopy, were unsuccessful due to complete upper airway obstruction. In view of failed airway access, an emergency surgical tracheostomy was promptly performed by the pediatric surgical team under continued uteroplacental support. Effective ventilation was confirmed before complete delivery of the neonate, who was subsequently transferred to the neonatal intensive care unit. Maternal hemodynamics remained stable throughout the procedure. After completion of the EXIT phase, uterine tone was restored, and the patient was extubated uneventfully. The post-operative course was uncomplicated. EXIT differs fundamentally from routine cesarean section as fetal oxygenation depends entirely on uninterrupted placental circulation. Excessive hypotension during uterine relaxation can rapidly compromise fetal oxygen delivery. Nitroglycerine provides rapid and titratable uterine relaxation while allowing prompt restoration of uterine tone. Failed fetal airway access should prompt immediate transition to surgical tracheostomy without delay, as anesthesia management directly determines procedural success. Although EXIT procedures have been described previously, reports documenting failed fetal laryngoscopy followed by successful surgical tracheostomy under placental support remain limited, particularly from resource-limited settings. This case underscores the pivotal role of the anesthesiologist in ensuring adequate uterine relaxation, maintaining maternal hemodynamic stability, and preserving uteroplacental circulation during prolonged fetal airway rescue. The EXIT procedure represents one of the most challenging situations in obstetric anesthesia. Meticulous pre-operative planning, invasive monitoring, readiness for failed airway scenarios, and seamless multidisciplinary coordination are essential for favorable maternal and neonatal outcomes.
Title: Anesthetic management of an ex utero intrapartum treatment procedure for congenital high airway obstruction syndrome with failed fetal airway
Description:
Sir,Congenital high airway obstruction syndrome (CHAOS) is a rare and potentially fatal fetal condition resulting from complete or near-complete obstruction of the upper airway.
The condition is associated with severe neonatal respiratory compromise immediately after birth, making prompt airway establishment essential for survival.
The ex utero intrapartum treatment (EXIT) procedure allows continuation of uteroplacental circulation while the fetal airway is secured and has emerged as a life-saving intervention in such scenarios.
We report the anesthetic management of an EXIT procedure performed for a fetus diagnosed antenatally with CHAOS, highlighting the challenges encountered during failed fetal airway access and the critical role of anesthesia in maintaining placental perfusion.
A 30-year-old gravida 2, para 1 woman at 36 weeks of gestation was referred to our tertiary care center following antenatal imaging suggestive of CHAOS.
After multidisciplinary consultation involving obstetricians, anesthesiologists, pediatric surgeons, neonatologists, and radiologists, an elective EXIT procedure was planned.
In the operating theater, invasive arterial blood pressure monitoring was instituted before induction of anesthesia.
General anesthesia was administered to facilitate profound uterine relaxation and ensure optimal surgical exposure.
Anesthesia was maintained with high concentrations of volatile agents.
Maternal hemodynamic were supported using a phenylephrine infusion to preserve uteroplacental perfusion.
Controlled uterine relaxation during the EXIT phase was achieved with a titrated nitroglycerine infusion.
Following uterine incision, partial delivery of the fetal head and upper torso was performed while maintaining intact placental circulation.
Multiple attempts at fetal airway establishment, including direct laryngoscopy, were unsuccessful due to complete upper airway obstruction.
In view of failed airway access, an emergency surgical tracheostomy was promptly performed by the pediatric surgical team under continued uteroplacental support.
Effective ventilation was confirmed before complete delivery of the neonate, who was subsequently transferred to the neonatal intensive care unit.
Maternal hemodynamics remained stable throughout the procedure.
After completion of the EXIT phase, uterine tone was restored, and the patient was extubated uneventfully.
The post-operative course was uncomplicated.
EXIT differs fundamentally from routine cesarean section as fetal oxygenation depends entirely on uninterrupted placental circulation.
Excessive hypotension during uterine relaxation can rapidly compromise fetal oxygen delivery.
Nitroglycerine provides rapid and titratable uterine relaxation while allowing prompt restoration of uterine tone.
Failed fetal airway access should prompt immediate transition to surgical tracheostomy without delay, as anesthesia management directly determines procedural success.
Although EXIT procedures have been described previously, reports documenting failed fetal laryngoscopy followed by successful surgical tracheostomy under placental support remain limited, particularly from resource-limited settings.
This case underscores the pivotal role of the anesthesiologist in ensuring adequate uterine relaxation, maintaining maternal hemodynamic stability, and preserving uteroplacental circulation during prolonged fetal airway rescue.
The EXIT procedure represents one of the most challenging situations in obstetric anesthesia.
Meticulous pre-operative planning, invasive monitoring, readiness for failed airway scenarios, and seamless multidisciplinary coordination are essential for favorable maternal and neonatal outcomes.

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