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TWO DIFFICULT AIRWAYS AT ONCE: EXIT procedure for severe fetal micrognathia in a mother with Pierre- Robin syndrome
Abstract Number: SU-41
Abstract Type: Case Report/Case Series
Introduction: Pierre- Robin Syndrome (PRS) is syndrome characterized by cleft palate, retrognathia, micrognathia and glossoptosis leading to upper airway obstruction and difficult intubation. Here, we report the management of an, Ex Utero Intrapartum Therapy (EXIT), procedure for a fetus with severe micrognathia secondary to PRS in a mother with PRS.
Case: A 25 year old G2P1 female with PRS with micrognathia and a history of a tracheostomy presented at 36 weeks for evaluation for an EXIT procedure. She had a mallampati grade 4 airway with severe micrognathia, macroglossia, and a cleft soft palate. Also noted on exam is a well healed tracheostomy scar. Her fetus was noted to have severe micrognathia on ultrasound requiring an EXIT. The delivery plan was to attempt fetal endotracheal intubation with a planned tracheostomy if intubation failed while on placental circulation.
The EXIT procedure was performed under neuraxial anesthesia with a double catheter technique. A low thoracic (T10) epidural was placed, followed by a lumbar combined spinal epidural. Spinal medications included 12mg of hyperbaric bupivacaine, 15 mcg of fentanyl and 150mcg of morphine. This double catheter technique was chosen to provide anesthesia for a vertical surgical skin incision and to avoid emergent tracheal intubation in a parturient with a possible difficult airway.
An arterial line was placed to monitor hemodynamics throughout the case and a prophylactic phenylephrine infusion was used to prevent maternal hypotension. Fetal immobilization was achieved with a remifentanil infusion at a rate of a 0.2mcg/kg/min. An intravenous nitroglycerin infusion at 100 mcg/min was used to provide uterine relaxation. The fetus was monitored using doppler ultrasound and a warm saline infusion was used to maintain intrauterine volume after hysterotomy. The fetal head was delivered and pediatric ENT surgeon performed a direct laryngoscopy using a rigid bronchoscopy to successfully intubate the male fetus without the need for additional fetal anesthesia.
After the fetus was delivered, IV remifentanil and nitroglycerin were discontinued and an oxytocin infusion was initiated at a rate of 18 u/h. Post operatively, the thoracic epidural was used for analgesia, allowing mother to visit her son in the NICU.
Conclusion: An EXIT procedure requires specific anesthetic considerations for both the mother and fetus. At our institution the use of neuraxial anesthesia is the technique of choice for EXIT to airway procedures. In this case we modified our technique using a double catheter epidural technique to provide both intraoperative anesthesia and post-operative analgesia, while avoiding general anesthesia. This case provides further evidence that neuraxial anesthesia is a viable anesthetic technique for EXIT procedures even in high risk parturients.