///2009 Abstract Details
2009 Abstract Details2019-08-03T15:55:31-06:00

EXIT Procedure and Tracheostomy in an Infant with Partial Trisomy and Pierre Robin Sequence

Abstract Number: 102
Abstract Type: Case Report/Case Series

Charese D Dakhil MD1 ; Grace Shih MD2

Case: A 29 y/o WF, G4P2, with no significant PMH, had an ultrasound at 15 weeks EGA demonstrating mandibular hypoplasia. An amniocentesis performed at 18 weeks revealed 46,XX and partial trisomy 6p. The father reported multiple offspring within his extended family affected by this trisomy and several died at birth secondary to airway complications. The patient was followed by maternal fetal medicine with reassuring biophysical profiles and serial ultrasounds revealing clinodactyly, shortened leg bones, mandibular hypoplasia, and polyhydramnios.

At 34 5/7 weeks EGA, a repeat cesarean section and ex utero intrapartum treatment procedure was planned, in conjunction with ENT and neonatology, due to partial trisomy, associated Pierre Robin sequence, and family history of infant death due to airway problems. She was premedicated with metoclopramide and famotidine, positioned supine with left uterine displacement, and standard ASA monitors were placed. A rapid sequence induction and intubation was performed without difficulty. A 9 French right internal jugular introducer and 20g arterial line were successfully placed. Isoflurane was maintained at 2.0% end tidal concentration in 100% oxygen to sustain adequate uterine relaxtion and supplemented with phenylephrine for blood pressure augmentation. Delivery of the infants head and right arm was accomplished 12 minutes after incision, and a pulse oximeter was placed. The ENT physicians performed a direct laryngoscopy and flexible endoscopy but were unable to place an endotracheal tube. A tracheostomy was accomplished 26 minutes after delivery of the head and arm. The umbilical cord was clamped after confirmation of tracheostomy placement and the infant was successfully delivered. The isoflurane end tidal concentration was decreased to 0.5%, nitrous oxide was started, and phenylephrine was discontinued. Adequate uterine tone was achieved with oxytocin and estimated blood loss was 1000mL. The patients muscle relaxation was reversed and she was extubated uneventfully.

Discussion: In 1923, Pierre Robin described a series of findings associated with respiratory difficulty which consisted of micognathia, glossoptosis, and cleft palate. The EXIT procedure is a rare but often life saving surgical technique for the infant with a potentially difficult airway. It allows partial delivery of the infant, while maintaining oxygenation through uteroplacental circulation, in order to establish an adequate airway for survival prior to delivery. Uterine relaxation is required to prevent uterine contraction and placental separation with the potential for considerable blood loss. Maternal hemodynamics must also be maintained for sufficient uteroplacental blood flow. The EXIT procedure was a life saving technique for this infant with a parital trisomy and associated Pierre Robin sequence. It allowed sufficient time to secure this difficult airway with a tracheostomy without maternal complications.

SOAP 2009