Airway management during an EXIT procedure for a fetus with dysgnathia complex

Abstract

Nonsyndromal dysgnathia is a rare disorder with a probable genetic basis characterized by a hypoplastic or absent mandible (agnathia), microstomia, microglossia, and ear anomalies secondary to a defect in the ventral portion of the first branchial arch caused by defective neural crest migration or proliferation. Dysgnathic newborn infants often suffer fatal respiratory failure from airway obstruction. Nineteen children with isolated dysgnathia complex are described in the literature--six were stillborn, eight died shortly after birth, and only five survived infancy. All survivors required tracheostomy to maintain an airway. It is difficult to intubate the trachea of these children and early airway management planning is important. We report a neonate who presented with a prenatal ultrasound diagnosis of severe micrognathia, polyhydramnios and a family history of severe micrognathia. Airway management was achieved with fiberoptic intubation through a laryngeal mask airway (LMA) during an ex utero intrapartum treatment procedure. Fiberoptic intubation was hampered by copious amounts of amniotic fluid. This child and her sibling are the first two siblings with isolated dysgnathia complex to have survived infancy and provide further support for a genetic basis to this condition.

Obstetric hemorrhage during an exit procedure for severe fetal airway obstruction

“…5 In addition, previous reports have recommended intraoperative crystalloid infusion into the uterine cavity to prevent premature placental separation. [6][7][8] Based on the pre-operative USS findings for our case, the surgical team was confident that there was no risk of placental trauma related to the primary uterine incision, and, therefore, they felt that intraoperative placental mapping was not indicated. The surgical team also reported that uterine relaxation was adequate and that no evidence of placental separation was apparent during the period of placental support.…”

“…2,11,12 However, the successful use of lower concentrations of volatile anesthetics has also been reported in achieving adequate uterine relaxation (based on intraoperative surgical assessment). 6,8,13,14 In our case, the inspired concentration of sevoflurane was titrated before hysterotomy to achieve an adequate degree of uterine relaxation. Nitroglycerin was also used to optimize uterine relaxation prior to hysterotomy.…”

Case series: Combined spinal epidural anesthesia for Cesarean delivery andex utero intrapartum treatment procedure

“…This procedure's indications have grown to include a variety of pathologies including fetal neck masses, congenital high airway obstruction, and dysgnathia complex. [2][3][4][5][6][7] Management of the EXIT procedure requires extensive multidisciplinary planning and coordination involving the obstetric, anesthetic, ENT, and pediatric teams. In addition to the usual anesthetic considerations for any CD, specific aims for the EXIT procedure include attaining maximal uterine relaxation and fetal anesthesia.…”

“…Some institutions infuse crystalloid into the uterine cavity to preserve uterine volume to aid in preventing placental separation. 2,6,11 Fetal anesthesia may be supplemented with im opioids, ketamine, and muscle relaxants. 8 Epidural catheters have been placed for perioperative analgesia, but regional anesthesia has not been commonly used as the sole anesthetic modality.…”

“…While this procedure originally gained popularity for establishing a patent airway and removal of tracheal occluding plugs used for the treatment of congenital diaphragmatic hernia, 1 indications have now grown to include a variety of congenital airway obstructions. [2][3][4][5][6][7] A deep general anesthetic with halogenated agents has been the anesthetic technique of choice for EXIT procedures. The halogenated agents provide the necessary uterine relaxation for partial fetal delivery and preventing placental disruption while also providing fetal anesthesia.…”