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Scott Webb (Host): Congenital tracheal stenosis is a rare condition in which the cartilage support structure of the trachea can cause a narrowing of the airway. It's also one of the many conditions that the multidisciplinary team at the Pediatric Aerodigestive and Airway Reconstruction Center at Stanford Children's Health can help with. And joining me today for another great panel discussion is Dr. Douglas Sidell. He's an Associate Professor of Otolaryngology and Director of the Lucile Packard Children's Hospital Center for Pediatric Voice and Swallowing Disorders and Pediatric Aerodigestive and Airway Reconstruction.

And I'm also joined by Dr. Kara Meister. She's a Pediatric Head and Neck Surgeon at Stanford Children's Health in the Children's Thyroid Center. And finally, I'm joined today by Dr. Karthik Balakrishnan. He's an Associate Professor of Otolaryngology at Stanford University and Lucile Packard Children's Hospital. This is Peds Talks from Stanford Children's Health. I'm Scott Webb. And thank you all for joining me today, as we discuss congenital tracheal stenosis and Dr. Meister, I'm going to start with you. What are the general signs and symptoms that indicate a serious tracheal problem in a baby?

Dr. Kara Meister: there are many different signs and they can be subtle all the way to life-threatening. Some of it depends on how severe the stenosis is and how old is the child. So we can even diagnosis in utero on ultrasound when it's associated with, common co-morbidities. Once a child is born, we are looking for signs of respiratory distress.

Those can be at rest. They may. On the other hand only occur when a kid has a cold. So a child who gets an upper respiratory infection, that gets severely sick. Stridor us with labor breathing., that's suspicious for tracheal stenosis, children who have, Croup like symptoms that, aren't typical meaning that they occur before the age of six months or a year, or really just don't recover in the way that we typically see that would be suspicious.

And then sometimes it's more subtle and it may only be a small area of the windpipe that's affected. Those kids can actually go all the way into, elementary, or even their teenage years. And they may just have Difficulty with colds, noisy breathing with exercise, decreased exercise tolerance, et cetera.

There's then another, category of kids that really only become symptomatic when they have airway instrumentation. So the classic scenario there is you have a child that goes for procedure., there's a little bit of difficulty putting in the endotracheal tube and then they have a big problem, with a demon.

And difficulty ventilating. So that child we'd have a high suspicion for tracheal stenosis.

Host: Yeah, and it's good to know the red flags and Dr. Sidell, I think many people are familiar with congenital tracheal stenosis, but I want to have you remind us what is congenital tracheal stenosis? And how do children with tracheal stenosis come to our attention?

Douglas Sidell, MD (Guest): You know, I will build a little bit on what Kara brought forth already. But just as a reminder, you know, congenital tracheal stenosis, it's not the most common airway abnormality that we see here at Stanford Children's Health, but it's definitely out there. And as an airway center, we can't really stress enough how important it is for primary providers and sub-specialists to really consider it on the differential diagnosis when children are demonstrating those signs and symptoms like those that Kara just mentioned. Congenital tracheal stenosis though occurs when, during the prenatal development of the airway, the trachea itself forms inappropriately. So, instead of having these C shaped cartilage rings on the anterior airway and an expandable posterior airway, we have complete tracheal rings that form along some length of the trachea.

And this can be the entire trachea and even into one of the bronchi leading into the lungs or it can be a shorter segment of the trachea. And so not only are these cartilage rings complete because that's a problem in and of itself, but the airway through that segment of complete rings is often very small. So as a result and depending on really how narrow the airway is, children may present with airway symptoms you know, immediately or shortly after birth. And thy may be things like Kara said, noisy breathing, stridor, but they may also present in early childhood or infancy with things just maybe asthma like symptoms that are unresponsive to treatment. And they can also present later in childhood with things as simple as exercise intolerance.

And on occasion, we have kids who are just incidentally identified, you know, we're doing an imaging study or an airway evaluation for something else. And the patients are largely asymptomatic and we identify this. And some people out there, can potentially even reach into adulthood before the stenosis is identified, whether incidentally or intentionally. Under other circumstances, the opposite is true. There are children who can be suspected to have complete tracheal rings based on specific findings, for instance, on prenatal ultrasound. And we've seen that recently here at our institution. This is truly a broad spectrum of pathologies and one that has many different potential symptomatic profiles.

So, I think the important thing to keep in mind here is that really, you have to think about it as a possibility when you have any type of airway symptoms in the appropriate patient.

Host: Yeah, definitely. And I want to bring in the third member of our all-star team here this morning. Dr. Balakrishnan, what are some of the risk factors for tracheal stenosis?

Karthik Balakrishnan, MD (Guest): We don't fully understand why tracheal stenosis happens or at the exact genetic mechanism that causes it, but there are some pretty strong associations. There is a fairly strong association with congenital heart disease and specifically pulmonary artery sling where the left main pulmonary artery actually comes off the right main pulmonary artery and slings around the back of the trachea.

And about 65% of those children will also have tracheal stenosis. So, it's a pretty strong association and vice versa. Other things that can be associated are common genetic syndromes. So, Down's Syndrome actually has a pretty strong association with this. And then the Syndromic Craniosynostosis association, so Apert, Crouzon, Pfeiffer syndrome, those are typically associated with what we would call the tracheal sleeve, where the entire trachea is sort of one tube or tunnel of cartilage rather than complete rings, but the management and the presentation is often similar. And based on some series out there, more than 20% of those syndromic craniosynostosis children can have associated tracheal stenosis. So, those are some common risk factors.

Host: Yeah, and I appreciate you sharing those. This is a fascinating topic. And, Dr. Meister back to you, what are the treatment options for children with congenital tracheal stenosis and what kind of long-term outcomes can children expect after tracheal stenosis repair?

Dr. Kara Meister: let me start by saying that not every kid with tracheal stenosis. Treatment. If the stenosis is subtle limited, either in diameter or in severity, there are some children that can be managed,, with airway clearance therapy and the care of a really great pulmonologist, in children, you have more severe tracheal stenosis.

Surgical repair is the standard of care. And the, current operation is called a thoracic slide tracheal class. This is a really cool operation where we basically take a long skinny pipe and traded in for a short fat pipe. And the way that we do that is we,, open the trachea and zipping it in the front and the back, and basically . Turning ind into end on itself.

And we do this in coordination with a pediatric airway surgeon, and a cardiothoracic surgeon,, working in tandem to make that happen. We often will do this operation on cardiopulmonary bypass or ECMO, depending on whether or not the stenosis extends past the level of the thoracic trachea and into the Bronco. And then we really rely heavily on that. Colleagues in the cardiovascular ICU, to help us manage these kids. What I tell parents is, the most delicate part of this operation is getting a child safely to surgery and getting a child safely recovered from surgery. The operation itself while technically challenging, is really, second fiddle, to that excellent perioperative care.

And if we can get them through those,, delicate,, recovery periods these kids do great, they may require one or two, dilations or interval exams. Certainly we take a look to make sure that they're healing appropriately, but then they go on to have a normal, respiratory outlook.

Host: Yeah, I love that as such a great word. Cool. It seems so appropriate. It's very cool. And, Dr. Sidell, as we've discussed before, when you've been on, it's really the multidisciplinary approach at Stanford Children's Health that is the real key here and listening to Dr. Meister there and about bringing in the different members of the team, different specialists. I'd like to have you discuss the benefits of that multidisciplinary approach to both diagnosing and treating tracheal stenosis.

Douglas Sidell, MD (Guest): You're right, you know, at Stanford Children's Health, we are firm believers in good communication and multidisciplinary care. And I think that we really build on the strong relationships with our colleagues in cardiothoracic surgery and cardiac anesthesia, as well as cardiology, to do this type of work. And we work with them in the operating room and the cardiac intensive care unit every day. So, it's not really just a fleeting or whimsical process. And so when it comes to treating congenital tracheal stenosis, this can not be undervalued. And it's really a natural process for us because the system is in place.

We have a systematic approach that really relies on the expertise of everyone on each of those teams. For instance, each of the patients who has an airway anomaly, like congenital tracheal stenosis, particularly those that have concurrent cardiac abnormalities, they're reviewed at our weekly Surgical Conference at the Heart Center.

And the airway is evaluated by a member of our Pediatric Otolaryngology airway team, like Karthik, Kara, or myself, and when appropriate, the airway and any cardiac abnormalities are corrected in the operating room by one of us alongside a member of the Cardiothoracic Surgery team, such as Dr. Michael Ma or Elisabeth Martin, or Frank Hanley to name a few.

And so we do this together. It's not a situation where one person operates and the other person manages them postoperatively. We work together throughout the entire process. And so after surgery, we work with the cardiac ICU, to manage the children safely in the recovery period, which I believe is labor intensive, but equally, if not more important than the surgery.

And we do so with the individual needs of the patient in mind. But we also have protocols that we've developed for these patients that work well for our team. I personally believe that it's really the direct result of this kind of teamwork and the standardization of a really complex and resource intensive process and you know this is the reason that we had the excellent outcomes for the children with congenital tracheal stenosis here at Stanford Children's Health. And I'm proud to say this, but I'm more proud to see it. And it's great working with the teams together, and learning with them along the way.

Host: Dr. Balakrishnan if a child with tracheal stenosis, has already had an operation, but requires evaluation. Is that something that you see at your center?

Dr. Balakrishnan: Absolutely. As Dr. Meister pointed out, we typically will apply this operation slide trachoplasty for these patients, but really there's a whole host of different strategies we can use, which are both, in some cases, endoscopic interventions, sometimes open surgical options done by otolaryngology.

Sometimes open surgery done in collaboration with the cardiothoracic surgery team. And so depending on the child's current situation and what treatment they've had before and what their current symptoms and problems are, we can adapt those techniques. And we're very happy to see those patients. And it turns out that when you look at patients who have had a revision reconstruction for congenital tracheal stenosis, after having had a previous attempt, the outcomes are actually quite good in the hands of an expert team. And I think this is where that multidisciplinary approach really comes in handy because you can have a lot of different expert perspectives coming together to make one unified plan that gives the child the best chance to succeed. And hopefully not need further revisions after that. But we are certainly happy to see those patients.

Host: Dr. Meister, as we wrap up, if I have a patient who I'm concerned may have tracheal stenosis, how do I go about deciding whether or not to refer and how do I refer that patient, you know, specifically.

Kara Meister, MD (Guest): We are happy to see, any patient with a suspicion of, airway stenosis, whether it's at the level of the trachea or perhaps, Other airway, anomalies or concerns. So if a child has a stridor and any warning symptoms, meaning the stridor or the difficulty breathing may be getting worse, maybe getting more frequent is more severe. Certainly if they have comorbid, cardiac or pulmonary conditions, we're always happy to evaluate those children. If you're within the Stanford children's health network, it's easy to put in a referral to,, our aerodigestive team it has its own epic order, or a cardiothoracic team. Children can be triaged, either way.

If you're outside of the Stanford children's health network, You can go onto our website. And there's a button that says request an appointment, contact us that will generate an email that goes directly to our nurse coordinator and the director of the thoracic trachea plasty program. So we're really trying to internalize any confusion about referral.

We're happy to see every kid that you have concerns about and, we'll do our best to follow up with you and transition them back to your.

Host: That's fantastic. And I'm just loving these Airway Center podcasts that we're doing. So, hopefully we keep them coming. I want to thank you all for your time today. And your expertise and compassion. So thanks so much and y'all stay well.

Dr. Sidell: Thank you, Scott.

Dr. Balakrishnan: Thank you, Scott. You too.

Kara Meister, MD (Guest): Thank you.

Host: And for more information, go to aerodigestive.Stanfordchildren's.org. This is Peds Talks from Stanford Children's Health. I'm Scott Webb. Stay well, and we'll talk again next time.