Repairing a baby’s pulmonary artery sling and congenital tracheal stenosis requires experience and interdisciplinary teamwork.
Sometimes the most important catastrophes in a life are the averted ones. That’s surely the case so far for Lovlyn Maguffee, the energetic 1-year-old book-loving toddler who loves to explore the freedoms that come with having just learned to run.
“No one who sees her now would ever guess there’d been anything seriously wrong,” says her father, Dustin Maguffee. But he knows well how easily the world might have been very different for his family if a small detail on her 20-week echocardiogram had gone unnoticed, or if she’d ended up at a hospital less experienced at correcting the rare kind of airway defect that an ultrasound discovered.
Neither Dustin nor his wife, Ciera, were big worriers. They’d had one healthy, vital, beautiful child already, and they loved being parents. So, when they were called back for a second fetal echocardiogram, they didn’t think much about it. The cardiologist who read the first one said she wanted a closer look at something and needed to wait until the baby shifted to see it more clearly. That “something” was the left pulmonary artery, which, instead of passing normally in front of both the trachea and the esophagus, was passing between the two in a configuration called pulmonary artery sling. This blood vessel abnormality can compress the airway itself, but more than half of babies with pulmonary artery sling also have a life-threatening condition called congenital tracheal stenosis, a severe narrowing of the airway.
After the echocardiogram, Dustin and Ciera were called in. Although Ciera was only about halfway through her pregnancy, her baby might well need a significant intervention to ensure her survival, the doctor said. Their physician recommended that the Maguffees, who live in Santa Cruz, get in touch with doctors an hour’s drive north at the extraordinary Aerodigestive and Airway Reconstruction Center at Stanford Medicine Children’s Health. If your baby has tracheal stenosis, there is probably no better place to have it addressed than Stanford Medicine Children’s Health, the doctor told them.
The trachea, also known as the windpipe or airway, carries air from the mouth to the lungs. A normal trachea has tiny horseshoe-shaped pieces of cartilage that hold it open while also allowing it to expand on one side. A child like Lovy, with congenital tracheal stenosis, has O-shaped rings (called “complete” tracheal rings because they form a complete circle). Not only do these O-shaped rings make the airway smaller, but because they are rigid, they also keep it from expanding when it must. For a child with complete tracheal rings, even a small cold may cause inflammation or swelling that can block the airway.
“Not knowing about tracheal stenosis ahead of time can be life-threatening,” says Douglas Sidell, MD, director of the Aerodigestive and Airway Reconstruction Center. “Kids who go undetected may catch a common virus or cold and then, seemingly out of the blue, have significant airway obstruction. Likewise, if the stenosis were unrecognized and a baby with airway distress had a breathing tube placed into the trachea conventionally, that could—because the stenotic trachea is so narrow and inflexible—cause a life-threatening disaster.
“Just knowing about the potential for tracheal stenosis in the setting of the pulmonary artery sling is important. This was a very good pickup by the fetal cardiologist,” says Dr. Sidell.
Preparing for anything
For the next few months before delivery, all the Maguffees could do was watch and wait. But the Aerodigestive and Airway Reconstruction team was busy planning and preparing for the baby’s arrival.
“We arranged for her to be born here at Lucile Packard Children’s Hospital,” says Dr. Sidell, “just in case she had problems with her airway right after delivery, which a subset of kids with tracheal stenosis do.” To be safe, they were ready to go directly from delivery to arterial and airway surgery in a matter of hours.
If the baby can breathe without difficulty at birth, though, it is often better to wait several months before performing the blood vessel and airway surgery, says Dr. Sidell.
Numerous things combine to make Stanford Medicine Children’s Health such an extraordinary place to address a pulmonary artery sling and complete tracheal rings. For one, the team sees more of these patients than any other dedicated center on the West Coast. For another, Stanford Medicine Children’s Health has a dedicated Thoracic Tracheoplasty Care Team, composed of an extraordinarily accomplished cardiothoracic surgery team and otolaryngology airway team, as well as a world-renowned Cardiovascular Intensive Care Unit (CVICU). “We know very well how these airway abnormalities intertwine with the heart and vascular problems,” says Dr. Sidell.
Smooth delivery
Ciera’s delivery went smoothly, and her baby, christened Lovlyn and quickly nicknamed Lovy, was breathing fine on her own at birth. Although a CT scan and an echocardiogram soon confirmed that she had a much narrower than normal airway, Lovy was otherwise healthy and full of beans. But in her tiny trachea an accident was waiting to happen.
“Even with a healthy baby you’ve got a heightened awareness,” says Lovy’s dad, Dustin. “But with Lovy, we were constantly checking: Was she breathing? Was there anything nearby that she might swallow? Was she sleeping? Was she OK?”
Dustin and Ciera had Dr. Sidell’s cell phone number and could call or text anytime questions arose. “That was tremendously comforting,” says Dustin.
For the next four months, the airway team followed Lovlyn from a safe distance (because of COVID-19 they relied mainly on video exams and conferencing) and made sure she was growing well and breathing freely.
The repair: slide tracheoplasty
The preferred surgical approach to repairing airways like Lovy’s is called slide tracheoplasty. The operation, combined with one for fixing the pulmonary artery, requires general anesthesia, intubation, and a pulmonary bypass machine. If you know the airway is very narrow, says Dr. Sidell, you don’t want to wait any longer than necessary to do the repair; the narrow and inflexible airway poses a daily risk.
“We want to fix the airway as quickly as we can, but we don’t want to do the heart surgery when it’s going to be more complicated or have a higher associated risk, as is often the case with infants in the first weeks of life,” says Dr. Sidell. “We’re looking for the optimal point where those two lines cross.”
In August, Lovy had what is called a microdirect laryngoscopy and bronchoscopy (MDLB), a procedure that uses a small camera to evaluate the entire airway. It confirmed that Lovy’s tracheal rings were indeed complete and that “her trachea was even narrower than we had suspected, given how well she was doing clinically,” says Dr. Sidell.
“When Dr. Sidell told me that the inside of Lovy’s trachea was the size of a spaghetti noodle, I thought about how hard it is for many people just to get spaghetti to wrap around a fork,” says Dustin. “And he’s got to do this delicate surgery on it. It still blows my mind!”
At birth, the narrowest portion of the typical healthy airway is at least 4 mm in diameter. Lovy’s trachea, on the other hand, was less than 2 mm, and her complete tracheal rings ran almost all the way from her voice box down to the bottom of her trachea. Slide tracheoplasty efficiently opens the complete tracheal rings and widens the trachea, so the repaired airway is both expanded and more flexible.
The eight-hour surgery went smoothly. And afterward the two surgical teams brought Lovy to the intensive care unit and transferred her to the extraordinary team there. “The expertise and amazing attention of these providers are key to our success,” says Dr. Sidell. They made sure Lovy was safe and comfortable and recovering on schedule.
“The ICU team showed us what to do and made us feel comfortable caring for Lovy ourselves after her surgery,” says Dustin. “She never really missed a beat from before surgery to after. Sure, it was a rough couple of weeks, but there was no difference in her attitude or spirit.
“A lot of parents must feel this way,” adds Dustin, “but Dr. Sidell will always be a big part of our lives, not just because of what he did but also because of how he made us feel through such a difficult time. He was always so capable and comforting. So reassuring.”
Today, at 1 year old, “Lovy is wildly energetic and yet as gentle and sweet as could be,” says her mom, Ciera. “She lives life to the fullest—you can tell she’s full of gratitude.” She loves the beach, the river, the sand, and giggling alongside her 5-year-old sister, Omie. But lately she might be happiest riding in the kid’s seat on the front of her mom’s bike. “She wears a big, beautiful, excited smile, and together we just breathe in huge gulps of fresh air,” says Ciera.
The whole loving family breathes a bit easier now, although when they hug her, they are still careful not to squeeze Lovy quite as tightly as they long to. That is, if they can catch her at all!
Learn more at http://aerodigestive.stanfordchildrens.org >
