Intricate planning helped protect two lives after early discovery of an airway defect.
Kara Meister, MD, an ear, nose, and throat (ENT) surgeon specializing in pediatric airway surgery, was in her Los Gatos clinic when she got the call. She sped to the hospital in record time, then jogged into the operating room and, still in her heels, scrubbed up. She was one of many. Earlier that afternoon, when Susan Hintz, MD, medical director of the Fetal and Pregnancy Health Program, received word that the care team’s patient Shelia had gone into labor three weeks early, it triggered a phone tree alerting about 40 different health care providers in 12 different subspecialties from around Lucile Packard Children’s Hospital Stanford and Stanford Hospital, all of whom were on call for the delivery and neonatal operation. For weeks, Dr. Hintz and her colleagues had been meticulously coordinating and planning for this day.
They were all assembling for a complex, multistage procedure that would usher Shelia’s baby safely into the world. Five months into her pregnancy, a sonogram had revealed that the baby’s airway was pinched and dangerously narrow. Subsequent tests showed that the baby had a condition called Pierre Robin sequence (PRS). Because it can cause the lower jaw to grow more slowly than the upper, an anatomical domino effect can result in a narrow airway, and babies with PRS are sometimes unable to breathe on their own once they are born. To ensure that she would survive delivery, Shelia’s baby would need a breathing tube to be placed into her airway before her umbilical cord was cut.
The complex procedure is known as EXIT-to-airway. EXIT is an acronym for “ex utero intrapartum treatment” (ex utero is Latin for “outside the uterus,” so EXIT stands for “treatment outside the uterus” during delivery). The three-part procedure entails, first, the partial delivery of the baby by cesarean so that the ENT surgeons have access to the airway. Second, the baby’s airway is very quickly secured—with a breathing tube or tracheostomy—so that she can still get oxygen once the umbilical cord is cut. Third, the delivery is completed and the cesarean is repaired. The procedure requires separate surgical teams for the mother and the baby. Other specialists, equipment, and resources must be on hand and ready for anything that might go wrong for either patient.
Months earlier, at Shelia’s second ultrasound, specialists detected her fetus’s abnormally small lower jaw and realized that it could endanger the baby’s life at birth. “Catching that so early was important,” said Yair Blumenfeld, MD, director of the Fetal Therapy Program. “Examination of the whole airway and the lower part of the jaw and neck are key to our routine fetal anatomy ultrasounds so that we can diagnose this type of malformation at the earliest possible time during pregnancy,” he said. “To prepare for the delivery, the team needs to precisely describe the problem and find any genetic syndromes or additional malformations that might be associated with it. With our multidisciplinary reviews, we look at every case from every angle.” The team not only made a plan for the EXIT-to-airway procedure but also began to ready everyone for the best course toward a healthy infancy and childhood once the baby was born.
Early labor is common in pregnancies complicated by fetal airway and swallowing problems, and although Shelia’s EXIT had been scheduled for close to her due date, she went into labor three weeks early. Fortunately, with the help of the Fetal and Pregnancy Health Program a few weeks previously, Shelia’s family had temporarily relocated to Palo Alto from their home about an hour and a half southeast of Stanford. It would have been unsafe for Shelia and her baby to have the delivery anywhere that wasn’t equipped and ready to do an EXIT procedure on short notice and did not have the multidisciplinary team to provide the coordinated complex care for both mother and baby.
Exceptional Lucile Packard Children’s Hospital Stanford team at Stanford Medicine Children’s Health
Packard Children’s Hospital is one of the few medical centers that can offer EXIT procedures. This is in part due to the integrated, multidisciplinary care that can be coordinated by the Fetal and Pregnancy Health Program, provided by an outstanding team of experts including maternal-fetal medicine, pediatric ENT, obstetrics and pediatric anesthesia, and neonatology, which is ranked third in the nation by the U.S. News & World Report Best Children’s Hospitals rankings for 2020–2021.
“It’s a monumental team effort planning for the procedure. Numerous adult and neonatal specialists must be committed and dedicated, and come together, sometimes on a moment’s notice in the case of premature labor. And there are very few places that a complex, coordinated procedure like this could be undertaken,” said Dr. Hintz, the coordinator for EXIT procedures. “Because the children’s hospital is attached to its adult counterpart, we can assemble all of the experts from all of the subspecialties that might be needed to address any crisis that could arise with either mom or her baby,” she said.
In the first stage of the procedure, overseen by Dr. Blumenfeld, the baby’s head and neck and one shoulder were delivered through a cesarean incision, requiring a highly specialized approach to maternal anesthesia and obstetric care that allows the baby to remain connected to the umbilical cord on placental support. Once Shelia’s baby was partially outside of her body, the ENT fetal airway team moved forward, led by Dr. Meister and Mai Thy Truong, MD. “The ENT team only has a few minutes to secure the airway. The baby is very slippery, and the mother’s uterus continues to contract, creating waves of movement,” Dr. Meister said.
While multiple teams were keeping mom safe and guarding the integrity of the placenta and the umbilical cord—the baby’s lifeline—Dr. Meister inserted a breathing tube into Catalina’s mouth and down into her trachea using an endoscopic camera while Dr. Truong was ready for a possible tracheostomy. Once the airway was secure, Dr. Meister’s group transferred the baby to the ICU crib while the surgical teams completed the delivery. When the umbilical cord was finally cut, the baby began breathing on her own. The coordinated EXIT from the womb—and the baby’s entrance into the world—were both successful!
Shelia awoke at 11 p.m., about five hours after she had been put to sleep. The next day, when Shelia, her husband, Ivan, and the baby were all finally able to be together in the NICU, they felt one powerful emotion, said Ivan: “Relief!” Shelia and Ivan named their new baby Catalina but almost immediately nicknamed her CC.
So that CC’s breathing tube could safely be removed, she still required a procedure called osteodistraction, which works something like braces or a palate expander, but for the lower jaw. Working closely with the rest of CC’s care team, plastic surgeon Derrick Wan, MD, implanted screws, which exerted constant, gentle, back-to-front pressure and stretched CC’s lower jaw so that it better fit her upper one. The procedure also opened up her airway so that she could breathe safely and normally without a breathing tube.
When CC’s breathing tube was finally removed, it was the first time she could cry or make sounds with her voice. “It was so good to be able to hear her,” said Shelia. “Everything improved a lot from there. We were able to hold her and to try breastfeeding and to teach her to use a bottle and a pacifier to stimulate her oral motor skills.”
But with the removal of the breathing tube, a new symptom emerged. Associated with her PRS, CC had laryngomalacia, a very loud, wheezy, high-pitched stridor caused by loose tissue above the vocal cords falling into the airway and vibrating with each breath. When she was 4 months old, CC went back into the hospital, and Dr. Meister conducted a microscopic surgical procedure, called a supraglottoplasty, that repaired the laryngomalacia. Afterward, CC could breathe freely, and, said Shelia, she began sleeping better, eating, and playing.
Today CC is a happy 1-year-old, playing with her brothers and sister, eating normally, beginning to talk, and looking forward to the adventures of a full childhood. She faces challenges; she will have cleft palate surgery in a couple of months, and her parents and doctors will closely track development of her eyes and ears. But that all seems surmountable to Shelia and Ivan, who plan to stay in close driving range of Lucile Packard Children’s Hospital Stanford. They know that CC’s coordinated, multidisciplinary team is among the best in the world and that each of the team members is dedicated to CC’s welfare and to smoothing any obstacles to her health or development that might arise.
Learn more: https://stanfordchildrens.org/en/service/ear-nose-throat
