When an expectant mother went in for a prenatal ultrasound at 32 weeks’ gestation, a large neck mass was identified. UT Southwestern physicians at Children’s Health℠, Children’s Medical Center Dallas and Parkland Health and Hospital System in Dallas determined a large mass, either a germ cell tumor or a vascular lymphatic malformation, was compressing the baby’s trachea. The imaging was referred to the ex-utero intrapartum treatment (EXIT) team who determined that an EXIT procedure addressing the airway obstruction prior to delivery was appropriate.
Fortunately, UT Southwestern physicians at Children’s Health and Parkland Health have performed EXIT procedures since 2007 and have developed an approach that ensures the complicated procedure — which involves a team of 30+ physicians and medical staff — goes as smoothly as possible.
“An EXIT is a complicated procedure. The team must simultaneously manage multiple health considerations for the mother and the baby and needs to be ready for multiple issues related to both patients,” says Sushmita Yallapragada, M.D., MSc, FAAP, Medical Director of the FETAL Center at Children’s Health℠ and Assistant Professor of Pediatrics at UT Southwestern. “We’ve built the expertise where we can manage those potentialities in a way that gives mothers and their babies the best opportunity for excellent outcomes.”
Multidisciplinary Evaluation of EXIT Procedure Candidates
At Children’s Health and Parkland Health, the first step toward a successful EXIT is a multidisciplinary meeting where maternal fetal medicine specialists, surgeons and neonatologists review the case. The team evaluates whether the baby is an appropriate candidate, while weighing medical considerations that might also complicate the mother’s well-being or threaten her future fertility.
“The meeting gives everyone a chance to ask questions and offer input,” says Patricia Santiago-Munoz, M.D., a high-risk pregnancy specialist and Associate Professor at UT Southwestern. “For every EXIT we perform, an additional three to four cases referred to the EXIT team are determined, following physician-to-physician consultations, to not require an EXIT.”
Dr. Santiago-Munoz and Dr. Yallapragada evaluated the most recent case.
In this case, the mother had safely delivered two previous babies and was otherwise healthy. Despite the airway obstruction, advanced prenatal imaging suggested the fetus’s lungs were developing appropriately.
“The mother’s health didn’t raise any concerns and, since everything with the baby was normal — her jaw, her chromosomes, and all of her other essential organs — we decided to move forward with the EXIT procedure,” Dr. Yallapragada says.
Teamwork is Key
An EXIT potentially combines multiple procedures, including intubation, tracheostomy, neck mass resection, chest tube placement, central line placement and thoracotomy, into a single procedure. And because this baby’s mass was found late in pregnancy, the team had limited time to prepare. Fortunately, the EXIT team and has expertise coordinating the procedure.
“There’s a significant amount of equipment and personnel that need to be managed and organized so everything’s ready at a moment’s notice,” he says. “And the team needs to be prepared to work together seamlessly in a variety of potential situations.”
For the latest EXIT case, the team was guided through three simulated procedures, during which every team member was in the operating room to rehearse even the smallest details.
The simulations ran through all possible scenarios:
- If direct control of the airway by intubation could not be accomplished, Romaine Johnson, M.D., Pediatric Otolaryngologist at Children’s Health and Associate Professor at UT Southwestern, would step in with a bronchoscope or glide scope armed with an endotracheal tube.
- If the mass caused such significant airway compression or deviation that intubation was impossible, Dr. Johnson would resect the mass and obtain an airway either by an endotracheal tube or by tracheostomy.
EXIT Procedure in Under 30 Minutes
The EXIT team was on call 24/7 for two weeks prior to the scheduled procedure in case premature labor occurred. Early on the morning of September 25th, it was time.
The OB anesthesia team placed the mother under general anesthesia, which calls for an extra layer of precision and caution during an EXIT. The anesthesiologist must sedate the mother and provide relaxation of the uterus. The degree of uterine relaxation has to be such as to prevent the uterus from contracting and prematurely delivering the placenta during the EXIT.
At 7:42 a. m., the team delivered the baby girl’s head and shoulders, keeping her lower body within the placenta. The fetus receives oxygenated blood via the umbilical cord — using the mother’s uteroplacental circulation as refers to as “the world’s most efficient heart-lung machine ever devised.”
Despite being unable to directly visualize the deviated airway with the laryngoscope, the team was able to obtain an airway intubating the baby via bronchoscope assisted by gentle retraction of the mass away from the airway.
Dr. Yallapragada was scrubbed in during the procedure to make sure the baby was doing well. After three comprehensive exams, Dr. Santiago-Munoz clamped the cord and fully delivered the baby at 8:03 a. m.
“Our neonatal resuscitation team stabilized the infant and put in IV lines prior to transferring her to the Children’s Health NICU for additional imaging and surgical repair,” Dr. Yallapragada says. “We were ready for anything, but this particular procedure ended up being quite straightforward.”
A Healthy Outcome
The mother’s recovery was smooth. The baby’s tumor was resected 48 hours after birth at Children’s Health.
The baby girl was home breathing and feeding normally just two weeks later. Pathologists determined the mass to be an immature teratoma. The mass had been completely excised. Additional specialists from pediatric oncology at Children’s Health determined that additional treatments would not be needed.
“For the EXIT to work, every provider needs to be at the top of their game,” Dr. Yallapragada says. “Expert imaging, precise coordination from multiple specialists and subspecialists, and supportive care for mom as we prepared for delivery led to a truly successful outcome. The mother recently checked in with us, and both she and baby are doing very well.”
In addition to the EXIT procedure, world-renowned experts from UT Southwestern at Children’s Health have established specialized Neonatal-Perinatal Medicine teams for complex conditions in the Level IV NICU as well as caring for babies until their 5th birthday through the Thrive Program. That’s just one of the many reasons why U.S. News & World Report ranked Children’s Health among the top 20 neonatal programs of 2019-20.
