Airway surgery makes Lovy breathe easier
Repairing a baby’s pulmonary artery sling and congenital tracheal stenosis requires experience and interdisciplinary teamwork.
Sometimes the most important disasters in a lifetime are those avoided. That’s surely the case so far for Lovlyn Maguffee, the energetic one-year-old who loves books and enjoys exploring the freedoms that come with learning to run.
“No one who sees her now would ever guess that there was something wrong,” says her father, Dustin Maguffee. But he knows how easily the world could have been very different for her family if a little detail on her 20-week echocardiogram had gone unnoticed, or if she had ended up in a less experienced hospital to correct the rare type of defect. respiratory tract. that an ultrasound discovered.
Neither Dustin nor his wife, Ciera, were very worried. They had already had a healthy, vital and beautiful child, and they loved to be parents. So when they were called back for a second fetal echocardiogram, they didn’t give it much thought. The cardiologist who read the first said she wanted to see something closer and had to wait for the baby to move 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, passed between the two in a configuration called the pulmonary artery sling. This blood vessel defect can compress the airways themselves, but more than half of babies with a pulmonary artery sling also have a life-threatening condition called congenital tracheal stenosis, a severe narrowing of the airways.
After the echocardiogram, Dustin and Ciera were called. Although Ciera was only halfway through her pregnancy, her baby may well need major intervention to ensure her survival, the doctor said. Their doctor recommended the Maguffees, who live in Santa Cruz, to contact doctors an hour’s drive north of the extraordinary Aerodigestive and Airway Reconstruction Center at Stanford Children’s Health. If your baby has tracheal stenosis, there’s probably no better place to get it treated than Stanford Children’s Health, the doctor told them.
The windpipe, also known as the windpipe or airway, carries air from the mouth to the lungs. A normal trachea has tiny pieces of horseshoe-shaped cartilage that hold it open while 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). These O-shaped rings not only make the airways smaller, but because they are rigid, they also prevent it from expanding when needed. For a child with full tracheal rings, even a small cold can cause inflammation or swelling that can block the airways.
“Not knowing about tracheal stenosis ahead of time can be life threatening,” says Douglas Sidell, MD, director of the Aerodigestive and Airway Reconstruction Center. “Children who go undetected can catch a virus or a common cold and then, seemingly out of the blue, have severe airway obstruction. Likewise, if the stenosis was not recognized and a baby in respiratory distress had a breathing tube placed in the trachea in a conventional manner, it could – because the stenotic trachea is so narrow and inflexible – cause a potentially fatal disaster.
“Just knowing the potential for tracheal stenosis in the framework of the pulmonary artery sling is important. It was a great idea from the fetal cardiologist, ”says Dr Sidell.
Prepare for anything
For the few months leading up to the 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 the Lucile Packard Children’s Hospital,” says Dr Sidell, “just in case she had problems with her airways right after giving birth, which she is. for a subset of children with tracheal stenosis ”. To be on the safe side, they were ready to go straight from childbirth to arterial and airway surgery within hours.
If the baby can breathe without difficulty at birth, however, it’s often best to wait several months before having the surgery on the blood vessels and airways, says Dr Sidell.
There are many things that combine to make Stanford Children’s Health such an amazing place to treat a pulmonary artery sling and complete tracheal rings. On the one hand, the team sees more of these patients than any other dedicated center on the West Coast. On the other hand, Stanford Children’s Health has a team dedicated to thoracic tracheoplasty care, consisting of a cardiothoracic surgery team and an extraordinarily accomplished airway otolaryngology team, as well as a care unit. World-renowned cardiovascular intensive (CVICU). “We know very well how these airway abnormalities intertwine with heart and vascular problems,” says Dr Sidell.
Ciera’s delivery went well and her baby, christened Lovlyn and quickly nicknamed Lovy, was breathing well on her own at birth. Although a CT scan and echocardiogram quickly confirmed that she had much narrower airways than normal, Lovy was otherwise healthy and full of beans. But in his tiny windpipe, an accident was waiting to happen.
“Even with a healthy baby, you have heightened awareness,” says Lovy’s father, Dustin. “But with Lovy, we were constantly checking: was she breathing? Was there something nearby that she could swallow? Was she asleep? Was she okay?
Dustin and Ciera had Dr Sidell’s cell phone number and could call or text whenever questions arose. “It was extremely heartwarming,” says Dustin.
For the next four months, the Airway team followed Lovlyn from a safe distance (due to COVID-19, she mainly relied on video reviews and lectures) and made sure that she was growing well and breathing freely.
Repair: slide tracheoplasty
The preferred surgical approach to repairing airways like Lovy’s is called slide tracheoplasty. The operation, combined with that of fixing the pulmonary artery, requires general anesthesia, intubation and an extracorporeal circulation device. If you know the airways are very narrow, says Dr. Sidell, you don’t want to wait any longer than necessary to make the repair; narrow and rigid airways pose a daily risk.
“We want to repair the airways as quickly as possible, but we don’t want to do 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 few weeks of life. life, ”says Dr Sidell. “We are looking for the optimal point where these two lines intersect.”
In August, Lovy underwent what’s called microdirect laryngoscopy and bronchoscopy (MDLB), a procedure that uses a small camera to assess the entire airway. This confirmed that Lovy’s tracheal rings were complete and that “her windpipe was even narrower than we had suspected, given her clinical condition,” says Dr Sidell.
“When Dr Sidell told me that the inside of Lovy’s windpipe was the size of a spaghetti noodle, I thought about how difficult it was for many people to just wrap spaghetti around it. a fork, ”says Dustin. “And he has to do this delicate operation on it. It always amazes me!
At birth, the narrowest part of a typical healthy airway is at least 4mm in diameter. Lovy’s trachea, on the other hand, was less than 2mm, and her entire tracheal rings stretched almost all the way down her voice box to the bottom of her windpipe. Slide tracheoplasty effectively opens the entire tracheal rings and widens the trachea, so that the repaired airways are both enlarged and more flexible.
The eight-hour operation went well. And subsequently, the two surgical teams brought Lovy to the intensive care unit and transferred her to the extraordinary team there. “The incredible expertise and attention of these suppliers is the key to our success,” says Dr Sidell. They made sure Lovy was safe and comfortable and recovered on time.
“The intensive care team showed us what to do and made us feel comfortable taking care of Lovy ourselves after her operation,” says Dustin. “She never really missed a beat from before surgery until after. Of course, it lasted for a few tough weeks, but there was no difference in his attitude or his mind.
“A lot of parents must feel this,” Dustin adds, “but Dr. Sidell will always be a big part of our lives, not only because of what he’s done, but also because of the way he’s made us. feeling such a difficult time. He’s always been so capable and heartwarming. So reassuring. “
Now at 1 year old, “Lovy is wildly energetic and yet as sweet and gentle as he can get,” says her mother, Ciera. “She is living her life to the fullest, you can tell she is full of gratitude.” She loves the beach, the river, the sand and laughing alongside her 5 year old sister, Omie. But lately, she’s perhaps the happiest to climb into the child seat on the front of her mom’s bike. “She’s wearing a big, beautiful, excited smile, and together we take in huge gulps of fresh air,” explains Ciera.
The whole loving family is breathing a little easier now, though when they hug her they’re still careful not to hug Lovy as hard as they want. That is, if they can catch it at all!
Learn more at http://aerodigestive.stanfordchildrens.org>