Tracheomalacia: Early Intervention Strategies for Lifelong Outcomes

Dr. Ali Mokdad and Dr. Krishna Pancham discuss multidisciplinary tracheomalacia care, the critical importance of early diagnosis and integrated surgical-medical management for complex airway cases.

Meet the speakers

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Transcript

00:00:00

(Music up and under)

00:00:03

Host: Hello, and welcome to this edition of Cook Children's Doc Talk. It's a very busy day here in the Child Life Zone at Cook Children's. So you may hear a little background noise. Today, we're talking with the pediatric tracheomalacia team. Tracheomalacia is a very complex condition that can be very difficult to diagnose and treat, often masquerading as asthma, recurrent croup or bronchitis. Adding to the diagnostic challenges is that standard imaging provides no clues or evidence due to the condition being structural, not inflammatory. Finally, while not considered a rare condition, managing it can be challenging because it requires a multidisciplinary approach, and that's where we begin this podcast.

00:00:51

Host: Here to talk about the advancements happening in the tracheomalacia program at Cook Children's are Dr. Ali Mokdad and Dr. Krishna Pancham. Before we get started, let me introduce them. Recognized for his expertise in the surgical management of tracheomalacia, Dr. Mokdad oversees the Cook Children's Tracheomalacia Program, one of the few centers in the nation, performing advanced procedures such as posterior tracheopexy to correct this complex condition. Dr. Mokdad earned his medical degree at American University of Beirut and his residency at the University of Texas Southwestern, and completed his fellowship at Children's Wisconsin and Johns Hopkins All Children's Hospital. He is board-certified by the American Board of surgery in general surgery, surgery critical care, and pediatric surgery. His significant experience also includes the surgical management of esophageal diseases, congenital diaphragmatic hernia, and ECMO support. A dedicated academic, he has contributed to the field with over forty peer reviewed publications on these topics. His name appears in numerous medical publications, including Surgery, the Journal of Surgical Research and Pediatrics, with his works focusing on disparities in surgical care utilization, socioeconomic impacts on surgical outcomes in children, tumor biology and outcomes of surgical procedures in ventilator dependent children.

00:02:24

Host: Dr. Pancham completed medical school at Nil Ratan Sircar Medical College, one of Eastern India's oldest and most prestigious medical institutions. He completed residency training in pediatrics at Children's Hospital of Philadelphia and his fellowship training at Children's National Medical Center in Washington, DC. Dr. Pancham was on faculty at Kentucky Children's Hospital, University of Kentucky, where he led the pediatric pulmonary research team and served as the director of the bronchoscopy program. His clinical interests include chronic lung disease of prematurity, pediatric neuromuscular disorder, airway anomalies, cystic fibrosis and severe asthma. He has published research on viral airway inflammation in premature babies and in asthma, and in 2015 received the Henry Christian Young Investigator Award from the American Federation for Medical Research.

00:03:22

Host: Welcome.

00:03:23

Dr. Krishna Pancham: Thank you.

00:03:24

Dr. Ali Mokdad: Glad to be here.

00:03:25

Host: To get started, can you give a little background on the Tracehomalacia Program at Cook Children's and why it is so important?

00:03:33

Dr. Ali Mokdad: We've now established the Tracehomalacia Program here at Cook Children's around two years. I come from training where we took care of a lot of these kids with tracehomalacia and what I saw was there are surgical options for these kids that do not do well with medical therapy and with meeting Dr. Pancham over here, who had a significant interest in tracehomalacia, we sort of worked together to put together the program. This is sort of how things started, and we've been able to help a lot of kids so far, and with seeing the success of this procedure and the program itself, has helped us kind of continue. And now we're into our second year of this.

00:04:19

Dr. Krishna Pancham: Being a pediatric pulmonary physician, we are the front line when the patient comes with noisy breathing or any kind of difficulty in breathing and Cook Children's being a tertiary and quaternary center, where we see a lot of referral from all over Texas, as well as sometimes from other states, where the patients have chronic wheezing episodes, chronic coughing episodes. It's easy to, as you mentioned in the introduction, that it can mimic asthma, or it can mimic some other form of disease, but it's important to have understanding of tracheomalacia, because this is a condition which is very important to diagnose and manage correctly, because we start with ABC.

00:05:00

Dr. Krishna Pancham: So airways comes first and then breathing, and then the cardiac and circulations. So if we don't see where the airway has an issue or not, then that can be problematic. So, I always had an interest in tracheomalacia patients, because it's very important, because when you have issue with airways, and if you don't fix it, it affects the lower airways, which is bronchi, as well as the exchange of gas in the lungs. So when I first came to Cook Children's four years back, we started the bronchoscopy program here, where any patient with noisy breathing, any patient who have been to the ICU, we started looking into it more thoroughly, so we do a bronchoscopy if the patient comes to the ICU intubated and has recurrent respiratory issues in the past. And we found that the patient with airway issues have diagnosis of actual tracheomalacia. So, as Dr. Mokdad said, it starts with medical management, and if the medical management works, we are happy. But unfortunately, if it does not work, then we have to go for surgical option. Given Dr. Mokdad's surgical expertise, the posterior tracheopexy is a revolutionary aspect where we can help those patients struggling for many, many years with tracheomalacia, or for many months, for that matter, if they are very sick. And some of these patients, they go to ICU, and they have recurrent respiratory issues, lifelong.

00:06:27

Dr. Ali Mokdad: And the other issue that also pushed us to create a formal program for this is the fact that this operation is not performed in our region at all. So a lot of patients tend to go to the east coast to be taken care of when there's no other options for them and they're only offered, for example, a tracheostomy, or offered the "You'll grow out of it," sort of spiel. And I actually took care of two Cook Children's patients when I was in Florida, because that was the only way for them to be taken care of before I came over here. That's also one other big reason as to why we pushed with this and formalized it, to be able to help a lot of people and kids in the region over here with this issue.

00:07:16

Host: So I understand that tracheomalacia is a somewhat frequent condition. However, because it can mimic many pulmonary conditions, as we just listed, such as asthma or croup, it can be difficult to diagnose and therefore treat. Why is this and what are the long term risks to delayed diagnosis?

00:07:35

Dr. Krishna Pancham: Flexible bronchoscopy, and with a dynamic airway evaluation, is a gold standard for diagnosis or tracheomalacia, but it's an invasive procedure. A lot of times, if the patient comes to the hospital and they don't have pulmonary medicine or subspecialty care, like ENT for that matter, or surgical care, then they are labeled as chronic wheezer or asthma or patient with recurrent group and unless ... until they get a direct evaluation of the airways, then it's hard to diagnose. More so, the noninvasive imaging techniques like the CT scan or even the MRI, it's hard to diagnose, and there's a lot of missed cases even if we do a CT scan on those patients. So doing a direct bronchoscopy, or doing a flexible bronchoscopy is the key, and a lot of centers, they don't have that capability, and that's why it's sometimes under diagnosed or misdiagnosed.

00:08:28

Dr. Ali Mokdad: To add to that, I think the bottleneck in this disease process is to make that diagnosis, because it's not made by imaging, unless you do a bronchoscopy, and do the bronchoscopy in a very specific way, which is sort of a dynamic bronchoscopy, you would not be able to diagnose it. So that's kind of the bottleneck where you end up having a lot of kids that are being managed, like asthma or recurrent croup, although they may not be following what the expected pattern for those disease processes. And that's why we've seen kids that are seven- or eight-years-old that have been struggling with this for a very long time. Twelve years old, 15 years old, I think, has been the oldest kid that we have been taking care of, which has been managed as a recurrent sort of asthma, and things are not working, and that kid developed a lot of lung damage because of the recurrent infection. So going back to what are longterm problems, one big one is permanent lung damage, what's called bronchiectasis. That is at least from a medical standpoint. And the other part is a lot of mental health issues, a lot of quality of life problems, because these kids tend to have a lot of visits to the hospital, and they're not able to play around with their friends as they want to. They tend to be shielded a lot from going to groups and parties and things like that, because they're more prone to get sick. So their upbringing is definitely limited and restricted, and that has a big impact on the child. Has a big impact on the parents as well. So that's also a big part of that long-term impact if you don't make an early diagnosis and treat adequately.

00:10:24

Host: So another challenge is, of course, that care requires a multidisciplinary approach. How many specialties are involved, and how is that level of care coordinated across teams? And, maybe most importantly, what is the advantage to this approach and outcome for a child?

00:10:40

Dr. Ali Mokdad: Tracheomalacia is rarely isolated. It can be, but in a lot of times it's not, which means that there's a lot of other disease conditions that come with it that can also contribute to the patient's respiratory problems. Kids may have aspiration problems, upper airway issues, lung problems, genetic problems, esophagus problems, all sorts of things that can contribute. And because of that, the appropriate way of managing these kids takes a whole multispecialty program. It's not borne in isolation. To be able to appropriately manage the tracheomalacia and any other conditions that are present with it, and to be able to figure out is the tracheomalacia the biggest component that's contributing to the patient's respiratory issues? Is there something else that has a bigger impact, or a huge impact that needs to be dealt with? Should we address the aspiration first and then the tonic problem that's leading to the tracheomalacia? So, it's a huge multidisciplinary team, and Dr. Pancham can talk a little bit more about the aerodigestive clinic that we recently started, and we're hoping it's going to be a huge impact for these families as we take care of them.

00:11:57

Dr. Krishna Pancham: Trachea is developed from a common structure, which is a part of the ventral foregut, and it starts with embryologically. So let me explain to you why we need a multidisciplinary clinic, because it starts with the body. If you go back to the embryology, which the trachea comes from proximal foregut, where it divides into the trachea, and then it divides and it's a structure and the back part it goes to becomes an esophagus, which is the food pipe. So it's very, very intricate. So both trachea and the food pipe are closely related. So that's what Dr. Mokdad said, that out of these patients, they have not just the isolated trachea problem, but they can also have a problem with their esophagus. Just thinking about tracheomalacia and not looking into the surrounding structure is going to be, I will say, not a complete or thorough evaluation. First, when I came to Cook Children's, I realized that, unfortunately, we do not have a robust and comprehensive or multidisciplinary clinic where we can, not just treat the patient with tracheomalacia, but also other comorbidities which is associated with tracheomalacia, as Dr. Mokdad said. So we were in the process of developing a tracheomalacia program, along with air digestive clinic, where not only pulmonary will see this patient, not only surgery will see this patient, but also ear nose and throat doctor, as well as gastroenterology. The ear nose throat doctor is very important aspect, because a lot of times they can have aspiration, as Dr. Mokdad said, where there is a connection between the upper part of the trachea or the larynx and the esophagus or the food pipe, and those patients will have chronic aspiration. The GI aspect also becomes very important, because lot of times these patients will have motility issues and they can, recurrent, have aspiration. So ultimately, what happens is that lungs being get affected because of ongoing surrounding structure. And if we don't see this issue with a different lens, then we'll miss the part of this complex problem, as you mentioned. We recently started the aerodigestive program at Cook Children's, which I feel is going to be a great contribution to our hospital, as well as the family we cater and the community we serve.

00:14:14

Host: You had mentioned in an earlier discussion that tracheomalacia is more complex than many other conditions. How so ?

00:14:21

Dr. Ali Mokdad: I would say, apart from the diagnostic bottleneck that we just talked about, it is complex in the sense that it is associated with a lot of other conditions that can contribute to the respiratory problems. And sometimes figuring out which problems are the most impactful and contributory to the patient's recurrent respiratory infections is not straightforward. To say 50% of this is related to tracheal Malaysia, the other 20% related to the genetic issues. It's hard to put numbers on a lot of these kids. And these kids tend to have had chronic respiratory issues. The families have gone through a lot. So there's a medical aspect of the complexity. There's a social aspect of the complexity.

00:15:11

Dr. Ali Mokdad: The procedure itself also has some technical complexity, doing a posterior tracheopexy. A lot of these kids may have had prior chest operations. They may have a lot of anatomical variations that would require additional procedures. It's a painful and a long operation. It has certain potential impacts afterwards that we educate the families on. It's a whole process as to getting in, figuring out the right thing, doing the operation and getting the kid through. But we have seen that has been very successful in the correct patients, and it has been a life-changer for the patient and the family. So although it is complex, with a multidisciplinary team and an expert team that has been seeing a lot of these kids over a long time, kind of understanding more the nuances of what portion of the management is correct and how to proceed. I think we've been able to help a lot of kids, and we continue to learn, as we take care of more kids, how to treat them in the best way and to be as most successful as we want after managing them, whether with an operation, without an operation, but through the tracheomalacia program and the aerodigestive clinic.

00:16:28

Dr. Krishna Pancham: I want to add also that the tracheomalacia is not complex in the diagnosis. I mean, it can be diagnosed, but the fact is that its management is difficult. The complexity is because it has to be diagnosed in a correct way. It's a lot of times we have seen that patient has been referred from other centers or other places where they have done a bronchoscopy on those patients while they are intubated or where they are completely sedated. So usually on those patients, we at Cook Children's have developed a protocol where we do bronchoscopy under light sedation, so the patient is having spontaneous breath, or they have, with Dr. Mokdad, he induces a special way to induce cough, and then we can see more dynamic evaluation of airways. One of the challenging aspect is to diagnosis correct. The second part involves is how bad is the tracheomalacia? So diagnosis has to be stratified, and whether it's mild, moderate and severe. And why it is important, because management depends upon it also. So mild and moderate form, we can hope that medical management can help them. So we start them on some of the medication, like atrovent as well as bethanechol. These are some of the medications which we use to strengthen the back part of the trachea. But if it is a severe cases, then its challenge is that a lot of times the medical management doesn't work. And if that doesn't work, then we don't have any option. Then then we have to do either tracheostomy or give them like positive pressure ventilation in form of noninvasive or invasive.

So that being said, now we have a newer surgical technique, which Dr. Mokdad brought to Cook Children's, where he can tie the back part of the trachea to the spine and just basically stent it open, and then this patient will not have collapsing of the airways. So the problem of the tracheomalacia will ... basically ... will be solved. But it also involves other parts of the structure. So that's why it's better to look in a thorough lens, as just explained, that one part being solved, we have to also make sure that the other surrounding structure, like esophagus or the food pipes, as well as the upper part of the airways, the larynx itself, is working well.

00:18:39

Host: So let's talk a little bit about diagnosis. There is a common misconception that children will naturally outgrow tracheomalacia by age two, and as we've discussed, the earlier the diagnosis the better. So what types of clues should physicians look for, or begin to look for, and what are the steps or process of elimination in establishing a tracheomalacia diagnosis?

00:19:03

Dr. Krishna Pancham: I think it's very important for initial physician or pediatrician to look for the clues. And it's important because a lot of times they are missed or labeled as, "Oh, this. We'll outgrow it." So one other aspect is we see with tracheomalacia is that they have the noisy breathing, but it's little bit different from asthma, because these patients will have persistent wheezing, or even if not persistent wheezing, but when they are trying to do some activities or crying, for that matter, or they're turning blue. This is like a subtle findings when the parents comes and tells you that my child has turned blue when he cries, or she cries, or having spells like all of a sudden -- even without crying they can have blue spells. And those patients, we have to be suspicious of something else it might be going on, and not just the periodic breathing, for that matter, or not just the asthma. Lot of times, these patients, they have a lot of reflux, so sometimes they will have hiccups, breathing faster than normal, recurrent infection. So that is one big thing. A lot of patients with tracheomalacia will come to the PCP with recurrent pneumonia as recurrent infection, and mom will be frustrated, or dad will be frustrated, like this is going on for so many times, so many issues there, where he's always having constant cough. So the other aspect is with aspiration or with tracheomalacia, is the cough and wheezing. So again, we have to be little more open [in] our aspect where we see that these patients with ... coming recurrently to the pediatrician or the PCP with problem, which is not making sense. So when you give them albuterol, for that matter, this patient with tracheomaalacia, it's important to remember that because it's albuterol, being a bronchodilator, it dilates the airways more and these tracheomalacia patients will have more issues, including the lower airways, the bronchus, and those patients will have more actually wheezing and more of problems. So if the patient is not responding to albuterol, that's another one big clue, which you have to keep in mind.

The other aspect is that, if these patients have recurrent infections, so the one particular part of the chest X-ray or has recurrent atelectasis with pneumonia in particular, like if you do the X ray and then that should raise a red flag that something else might be going on with the patient. The other aspect sometimes genetic syndrome, like DiGeorge patients and some of the illness patients, there's some of the other Down syndrome or Trisomy 21 patient. These patients, they are tracheoesophageal fistula patients. So these patients, when they have chronic cough, chronic wheezing episodes, these genetic conditions are associated with tracheomalacia. So a pediatrician or even the ER physician, if they see these patients, they should raise the red flag, and they can we refer to pediatric pulmonary medicine, where we can help those patients by diagnosing so these are the subtle clues which can happen. The other clue, which I have noticed, is that a lot of reflux or hiccups, and particularly posture sometimes. So baby with tracheomalacia, they like to extend their neck because that's how they can breathe in a ... physiologically, it's got the least resistance so they can breathe easily. So these patients typically try to raise their necks in a position so that they can breathe easily. The grunting noises also is very important because, we call it auto-PEEP, that they want to close their glottis so that they can extend the air inside the lower airways. So these are the subtle signs where the patient will be coming with the grunting and pauses of the breath just to keep the airways inflated. So that is another subtle finding, which we see that. Patients, like older kids, typically, we see the recurrent croup is happening, or even in early phase of their life, that they have recurrent croups. So this is something which, yes, it could be asthma. I'm not saying that it cannot be asthma, but these patients can also have something else. So recurrent croup, recurrent infection, these are also the subtle signs where we have to think about something else might be going on.

00:22:52

Dr. Ali Mokdad: Yeah. I mean, it's all about the pediatricians that are the frontline to see a lot of these kids and to pick up these patterns that are not quite what they're expecting, whether it's a reflux problem, but it's not quite acting as they expect, or an asthma but not quite acting. And I'm sure a lot of pediatricians and family medicine doctors out there have those patients that they remember, that are frequent flyers, that they're just sort of just off, in some sense, with a lot of the criteria that Dr. Pancham talked about as clues as to, "Maybe there's something else going on that we need to dig into." To add, older kids that we've seen that have had issues with these relentless coughs, although they may not be an infection, but we've noticed some of these kids that are seven, eight year old, 10 year old, 12 year old, that say, "All right, they've had a cold, and then developed this cough, and this cough would just not go away." Relentless. Or when they start coughing, does not go away. And a lot of times, this has been related to tracheomalacia and the fact that the airway is collapsing and sensitizing their airway. So, it's not easy. A lot of it's subtle patterns that the pediatrician would need to be attentive to, to kind of guage that something else might be going on. And maybe a pulmonologist, or anybody with the ability to do a bronchoscopy might be helpful to look into if there's anything else going on that they might be missing.

00:24:20

Dr. Krishna Pancham: And to add further, we also ask, does tracheomalacia gets better with time? Yes, it gets better with the time. But that's not true in every ... in each patient. A lot of times we have patients where they have six years, seven years of age, has been struggling or recurrent respiratory issues and has been on multiple medications. I had one patient who had been on antibiotics more than 40 times in an entire lifetime, and he was just eight years old. And when I first did a bronchoscopy, for me it was not surprising, but the parents were very much surprised that this was not diagnosed for like, a past eight to 12 years and continues to have recurrent respiratory infection. The other aspect we see in the patient, where they come to our clinic, then we do a lung function test, and sometimes we can see subtle changes in the lung function, especially when they try to expire, or we call it expiratory flow, where there is a blunting of the flow there. Then we also think that it might be something else going on, and that usually is extra thoracic component, which is tracheomalacia part, where we feel that our intrathoracic depends upon the layer of obstruction, then we can also identify in the lung function test. So basically, what I'm trying to tell to all the listeners which who are listening to us is, keep your eyes open and see that if something else might be going on, and it's not following a pattern, if not following a pattern, we are here to help them.

00:25:43

Dr. Ali Mokdad: And since we're talking about diagnosis, the other difficulty of making a diagnosis is that somebody figured out that this patient is not quite following a pattern. Let's take a look into their airway, do a bronchoscopy and see if that's something that comes up here that can explain what's going on. And you can definitely miss tracheomalacia if you don't do what's called a dynamic bronchoscopy. Because tracheomalacia is a dynamic form of an obstruction, it's not a fixed form, in the sense that when you go in and take a look, you may not see any narrowing. It's only when you induce the patient to cough or increase their intrathoracic pressure is when you might see it. Now, in severe cases, it's very obvious, but there are these moderate cases where, unless you do a dynamic bronchoscopy, you may miss it, and that's not commonly done.

Traditionally, bronchoscopy is done. You sedate the patient, you go in, you take a look, check the airways, maybe do some lavage and take out some specimen. An appropriate dynamic bronchoscopy entails three phases, okay, that can be done with a flexible bronchoscope, that can be done with a rigid bronchoscope. But the important thing is, it has to entail these three phases to have a full and comprehensive assessment of the airway. Here at Cook Children's what we do is the patient is lightly sedated, and then we do our bronchoscopy. We evaluate the upper airways, but then we go into the trachea. We watch the airway while they're sort of shallow breathing while they're lightly sedated. We check the amount of secretions, the lining of the airway. We also check for any sort of how the airway is reacting to shallow breathing. Are we seeing any sort of obstruction? We look at the cartilage shape. We look at the posterior membrane. Is it wide? Is it normal? That's the first step. The next step is, while they're still lightly sedated, we induce cough. So we sort of tickle the airway. We have different ways of doing that, but that induces cough in the lightly sedated patient. And when they cough, they increase intrathoracic pressure, and this is how we can pick up, Are they having an obstruction related to their tracheomalacia? To what degree is it? Which portion of the airway is it involving? Is it the distal part of the airway? Is it the branches going to the right lung or the left lung?

So that's the other part. And then the third part of a dynamic bronchoscopy is we heavily sedate the kid at this point where we stop their breathing, and then we sort of distend their airway, and then we go look for any sort of abnormalities in their airway. Do they have any additional connection to their esophagus? Are the airways anatomically correct? Is there a branching that is different or abnormal in any sense? And we've had a lot of cases where you would go in, things look fine, and until you perform these different steps, you would not know. So sometimes a quick glance and quick look may not be enough and you may miss it. So that's the other part of diagnosis, picking up if there's something off. And if there's something off with anybody with a capability to do the bronchoscopy, especially if they're thinking about tracheomalacia or something like that, to do an appropriate dynamic bronchoscopy. And I think that's sort of a big part of the diagnosis component.

00:29:10

Host: It seems to me, then, the longer it takes to diagnose this, potentially, the more complications a child could develop over time as they grow?

00:29:19

Dr. Ali Mokdad: 100 percent. Now there are some kids that are born with severe tracheomalacia that up front you would know there's something wrong. These are the kids in the ICU and the NICU that are generally offered the tracheostomy tube. They cannot come off oxygen. They cannot come off the ventilator. The tricky ones are the ones that have these recurrent respiratory infection, recurrent croups, as we talked about, manage for reflux, managed for asthma. It's not quite right, but we still push through with it. And yes, we've seen kids at a later age that have developed permanent lung damage. We've seen kids that had to go on what's called ECMO, which is extracorporeal membrane oxygenation, sort of a bypass type thing, because the respiratory infection is so severe, and their tracheomalacia does not help them overcome that as easily, and they end up in an ICU. We've done at least two kids now that have went through that severe condition. And then you have the kids that have spent their entire childhood restricted of play. The parents have told the PE teacher to kind of let them lie down or kind of keep the activity pretty light because they may start coughing or not be able to continue. As I said, there's that social mental aspect. And there has been, actually a couple of papers at least that looked at mental issues in kids with tracheomalacia and teenagers, and they've seen a significant impact. And the impact, as I said, is on the kid. The impact is also on the family as well. So the more you push that down the line, the bigger the impact is on the kids and on the families.

00:31:00

Host: What are the comorbidities that can complicate diagnosis, and what role do they play in determining diagnostic tools? Is there a multidisciplinary consensus such as pulmonology and gastroenterology, et cetera?

00:31:13

Dr. Krishna Pancham: Tracheomalacia may not be isolated condition, and we need to have a comprehensive approach to look onto these patients condition. And as we just discussed, that a lot of these patients, they can have reflux, they can have problem with aspiration, they can have issue with the gut motility, also like where they have dysphagia, or the problem with the esophagus, where they cannot swallow it normally. And then that's an issue. And again, the problem is combination is because of they arrive from the same structure, embyrologically. To look into it again, it requires not only just pulmonary but the help from ENT friends or as well as GI, who happens to help us a lot. But before I came to

Cook Children's, I had experience in running aerodigestive program, so when I came here and I noticed that we don't have it, so my first interaction with our subspecialties, including GI, was to help to start an aerodigestive program so that we can provide care, not just for tracheomalacia, but a comprehensive care about other comorbidities condition they may have. Interestingly, Cook Children's being one of the 10 largest hospitals in the country, but we were the only center who did not have aerodigestive program. So we cater a large population in Fort Worth area and the surrounding counties. And unfortunately, we did not have the aerodigestive program as well as tracheomalacia program. So as Dr. Mokdad initially mentioned that he had two of my patients actually in Florida I had sent him to because I couldn't find anybody who can help this patient in Texas. So I have to send those patients to Florida to help get help. So it required time. It required, you know, team building, but ultimately, now we have a robust system where we can help and provide the care with this complexity, and not just the tracheomalacia, but the whole comorbidities condition the patient may have.

00:33:14

Dr. Ali Mokdad: Yeah, and because of a lot of these conditions that can, as I said, culminate to having the same respiratory problems, and try to figure out what is causing what, and which one is contributing the most, now we do something called a quadscope, quadruple scope, which means the four specialties do the scopes, as in pulmonology, surgery, ENT and GI. In one setting we do bronchoscopy, laryngoscopy, evaluation of the distal airways, as well as esophagoscopy, gastroscopy, all in one sitting, which is obviously helpful for the kids. So we are doing everything under one sedative. And the other big part of it is now you have information about all aspects that can cause these respiratory problems, and then you can make more informed decisions about what would be the most helpful management option going forward. But I think this is also a huge addition, to be able to have all these specialties perform their diagnostic scopes at the same time. And during multidisciplinary clinics, the aerodigestive clinic, to think about these issues all in one sitting and kind of jump ideas off of each other to be able to come up with the best plan.

00:34:26

Dr. Krishna Pancham: And a lot of times they have a problem with their speech. They have a problem with their nutrition. So now we have not only our force of specialty but, in addition to those, we have speech as well as nutrition to look into it. So again, this is a very comprehensive program here. One thing unique at aerodigestive program, which we have at Cook Children's, is that we have not only the surgery team who is going to evaluate the airways if a patient needs a tracheopexy, but also a GI specialist who deals with gut motilities, and so he's a motility expert. So that is also a huge addition, Dr. Mansi, Sherief. We are lucky to have him in our team who does GI care at the aerodigestive program at Cook Children's, and he helps those patients who have motility issues.

00:35:17

Host: So once you have a confirmed diagnosis, how do you determine the treatment options for a specific patient, such as surgery or pharmacology, and how do you begin the treatment plan?

00:35:28

Dr. Krishna Pancham: Once we diagnose the patient's condition, we look for not just the tracheomalacia, but some other comorbidities condition the patient may have. So sometimes we may have to refer those patients to, again, before arrive, we have to refer them to ENT, ear, nose and throat doctor, or to GI subspecialists, who have, again, a comprehensive plan built for those patients. So for isolated tracheomalacia, we start with medical management, especially to see if there is any improvement with those medical management, as I said earlier, we start with bethanechol, sometimes atrovent, or in combination with inhaled corticosteroid to reduce the inflammation in the lungs. And in certain cases, we have to sometimes give oxygen for those patients to help to bridge the gap when the patient is having acute respiratory infection, they may require oxygen, and unfortunately, they have to come to the ER or go to the hospital and get admitted. So in some cases, we may have to send you home on oxygen. So for those patients, we follow them very closely, especially in pulmonary medicine. We see them very often, every three months to every six months, and sometimes even every one month. The challenging part happens when some of these patients do not respond to the medical management, and despite our best effort, these patients are not getting better or continues to struggle with their breathing. And then we definitely involve our surgery team here to have them other aspect of management, which is the surgical option. I will let Dr. Mokdad speak about posterior trachopexy, which is a unique aspect of management of tracheomalacia.

00:37:01

Dr. Ali Mokdad: Yeah, the key, as I said, patient selection is very important in establishing sort of medical management and then kind of following whether this worked or not. It's just important to know if tracheomalacia. is causing the problem and when there's other comorbidities, to figure out how much contribution are those making, and whether those needs to be addressed first, or the tracheomalacia.. Now that we have the patient that we have determined tracheomalacia. is the primary culprit of their respiratory conditions and their symptomatology, and they have been trialed on pharmacological therapy, and they have continued to have persistent symptoms, or at least not well controlled. We discuss with the families surgical options. The surgical options that we talk about the predominant thing is something called a posterior tracheopexy. Although there's a lot of different aspects and techniques and additional things that may be involved, based on what we see in the anatomy. But to keep it simple, the idea is that in tracheomalacia, the backside of the trachea, the posterior membrane is generally the biggest contributing portion of the collapse of the airway. It's wide. It collapses with any time you increase intrathoracic pressure, and acting on that membrane is the key to addressing the obstruction and then the symptomatology. So typically, talking about a kid that does not have other significant morbidities, a kid that has not had previous chest surgery, then a posterior tracheopexy is typically done through the right chest. It's an incision of about two and a half three inches. And obviously depends on when dealing with a baby or a teenager, but we do access through the right chest, below the scapula.

I kind of tend to, when I see the patients, kind of go over a lot of pictures as to how this goes. And we have some videos up on the tracheomalacia web page that kind of goes in detail as to how we do the operation. But the idea is we secure the backside of the membrane to the backbone, or some of the ligaments of the spine. And the idea is that when you increase your interthoracic pressure, this membrane is secured, and it does not collapse anymore. In some kids, when the membrane is very wide, we have to excise a portion of that membrane to make a little bit narrower so it does not flop into the airway and cause collapse.

In general, for these patients, an operation would be four to six hours, as I said, if there's no other portions of the operation that need to happen, they are in the ICU after surgery. In general, we remove the breathing tube immediately after surgery, they tend to stay in the hospital. On our average, in our patient population, for that specific around three to four days they stay in the hospital. A lot of it is to kind of pain control and kind of come down on their oxygen, make sure they're doing okay. We tend to see them in around a month after their surgery, make sure everything is okay, and then we do what's called surveillance bronchoscopies around six months to kind of reevaluate the airway, see how they're doing unless they're having some recurrent issues. We may have to do that a little bit earlier, but that's typically how our protocol has been

00:40:22

Dr. Krishna Pancham: I also want to add that one of the unique aspects of tracheal program here at Cook Children's is that pulmonary surgery works very closely, where we're not just diagnosed the patient early on, but we also see them during their admission in the hospital, as well as close follow up. Other institutions, so far, to my knowledge, is that we have the surgery and the patient is done and done, but not here at Cook Children's. We try to follow them very closely, even after the posterior tracheopexy has been done, and give the thorough and comprehensive care post operatively also, so that if they have any issues, we can manage those things or those aspects or those conditions. So some of those patients, after the surgery, can still have some of the respiratory issues. So we make sure that these patients have ... are on the medication even after the surgery. And then we slowly wean them off, depending upon how they are doing. So that is also very important and unique program which our institution offers is that we also provide post operative care by doing surveillance bronchoscopy to see how much improvement they have if they have any post operative issues or complication, if they have issue with the esophagus, having dysphagia. So I can contact Dr. Mokdad and the GI team if needed, so that we can not only just done with the surgery, but also give the post operative care and follow up closely those patients. And once they are doing quite well, then they can be taken off all the medication they are [on]., And that would be just like a cure.

00:41:57

Dr. Ali Mokdad: Wanna add a couple of points here about this that are probably pertinent. So the first thing, and this is what we talk to families about after surgery or pharmacological therapy, when we initiate management for tracheomalacia. The goal is not to have the child not have an infection anymore. Obviously, because any kid has infection, the goal is to have less frequent infections, and when you have an infection, the kids will able to overcome that infection like any other kid over a few days, not requiring to come to a hospital for that. So sort of go back to what the normal cold respiratory infection is like. So that we talk about that. The other part I wanted to highlight is that if you go out into the surgical world and say tracheopexy, just a nuance to be more relevant to the surgeons, people have been doing tracheopexy For a long time. However, traditionally, when somebody says tracheopexy, they're doing something through the front portion of the trachea on the cartilage, not quite on the posterior membrane, like we talked about. So they go through the front and they pull up the trachea and do that. And the issue with that was what's called an anterior tracheopexy, where we're using the front side, which is traditionally what people think about when you talk about tracheopexy, that does not address the problem, which is the back side of the trachea. And doing that operation from the front side has not been very helpful for a lot of kids, and we've seen that. So that's sort of a nuance that sometimes may come up when somebody says tracheopexy. So ideally, it's a posterior tracheopexy that acts on the posterior membrane. Is the unique thing over here.

00:43:43

Host: Tracheomalacia has not been managed surgically, traditionally or historically, and that already makes this program very unique. Even more distinct is the utilization of posterior tracheoplexy for treatment. An approach that only three programs in the country, if I understand correctly, are currently taking so why have you chosen the surgical method as your standard for the surgical treatment of tracheomalacia, and what advantage does it afford your patients?

00:44:14

Dr. Ali Mokdad: As I said, Dr. Pancham, before posterior tracheopexy had been done at Cook Children's, was sending his kids either to Boston Children's or to All Children's in Florida, which is similar to a lot of other hospitals in the region. Aside from those two programs, there are possibly CHOP has a program. Otherwise, there's no fully established programs that do comprehensive tracheomalacia care and posterior tracheopexies. So this was a huge gap in this region. And given my experience with that and seeing that a lot of these patients have done well, and it has been life changing for a lot of kids, I think it was important to leverage that skill set and being part of a, umm, institution over here that has been at the forefront of innovation and doing things and putting resources toward things that they see as important, plus having Dr. Pancham with a lot of expertise and interest in tracheomalacia, I think, allowed this program to succeed. We're doing something that is not commonly done in the country. I know a lot of places are working towards that, which is helpful and very important. But the technique of the operation is not straightforward. You need to have some component of training, or have done it with some experts before, to be able to just jump in and do it. It's not one of those where you just read about it and kind of jump in and do it. There's more to it. and doing the operation itself is a very small part. The big part is the entire team and the work that happens before the operation and after the operation. As we talked about, patient selection is key. So having the expertise and figuring out which patient will do well and which patient will not do well and how to react to certain issues that come up after surgery and things like that, requires a lot of expertise and seeing a lot of these kids over time, and that's what we have here.

00:46:13

Dr. Krishna Pancham: And I also want to add that developing the tracheal leadership program involved not just, again, pulmonary or the surgery team, it involves the whole hospital care. So these patients who have undergone posterior tracheopexy or correction of the tracheomalacia, the whole team has to get involved. So when Dr. Mokdad came, he started introducing the tracheomalacia of surgery to the ICU. He talked to the pediatric ICU, then he's talked to the neonatal ICU, then he talked to the anesthesia department also. So why it is necessary is because these patients involves preoperative management and postoperative management. So by just doing the surgery is not just we are done with this. We want to educate, and we would like to make us a good team where anesthesia understands what Dr. Mokdad is doing, the PICU is understanding what they are doing and how to cater those patients and have minimal preoperative, postoperative complications or intraoperative complications. So that is very vital. Anesthesia now understands what tracheopexy involves, and what is the anesthesia they need. The pediatric ICU team they have. We have a protocol there. Then how these patients get better on the postoperative management, what we need to do postoperatively to help them. Also, pain team, who is under anesthesia, also gives a proper pain medication, which is important for a proper outcome. So just not doing the surgery is important, but also pre and post operative care is important because that reduces the complication associated with posterior apolloxy.

00:47:50

Host:Are these treatment options curative? Or do some types of diagnoses require ongoing care, which somewhat we've touched on throughout childhood, but even into adulthood, and if so, what kind of long term care can the patient and the patient's family expect, and what role does the child's pediatrician or primary care doctor play?

00:48:10

Dr. Ali Mokdad: Regarding whether it's a curative or not, that all depends on,, "Is this isolated tracheomalacia? Are there other related conditions that contribute to the patient's symptomatology?" In those cases, when it's isolated, tracheomalacia, and you fix the anatomic problem, it's curative, you're good. And this is generally the best patient population that will see the most benefit from this operation, because you fix the one thing that's causing their symptoms. Now, other patients have genetic issues, aspiration problems, motility problems, reflux problems, all sorts of other things that we had mentioned earlier. And helping with the tracheomalacia helps them, but it is not necessarily curative, because there are other aspects that are contributing to their symptoms. But fixing their tracheomalacia in a kid that has aspiration, for example, allows them to clear the secretions, whatever they aspirate. Now it's easier for them to clear it because they don't have an obstruction anymore, so that it doesn't stop the aspiration, but it makes it easier to overcome issues with aspiration, for example.

We try to talk to the families upfront about sort of expectations and things like that. So that's first part of that question. The other part regarding long term. This operation has not been done for a very long time, so less than 10 years. And the initial group up in Boston Children's, when they started working on it in the mid teens, they were still figuring out what works best and what techniques and what sutures and what worked, what does not work, until they sort of perfected that in a way that seems to help the kids the most. So there has not been very long term data about these kids, because that's a question for a lot of families. All right, so now you fix that backside of the trachea to the bone, what happens? My kid's three years old. What if they're 10 and 15? Is it going to come off? Are they going to be able to grow their trachea with it? Is it stuck? So we don't have very long term data about that. What we know is that we use absorbable sutures, so we count on the body to produce scarring to be able to maintain that fixation of the posterior membrane, which allows the trachea to grow. By doing surveillance bronchoscopies that we're doing, we're trying to get more data about how does the trachea react to this operation and what we see. And as part of the research component that we're doing with this program is to look at one part, what happens down the line as we follow these kids and kind of look at what happens to their trachea, what happens to their symptoms? Does it regress? Does it maintain it if it regresses in one patient population?

So that's one big aspect of the research that we're doing. And the other part of the research that we're doing is to figure out what sort of benefit do patients get from this procedure? We talked about isolated tracheomalacia is nearly curative for these kids, but the kids that have other comorbidities what to expect so we can inform families upfront, better. If you have a kid that has aspiration problems, and we know this is not going to get better, there's some sort of issue with the swallow function that you cannot fix, you're going to continue to have aspiration, but you fix the tracheomalacia.

What to expect after that? Who are the kids that we expect are going to do really well? Who are the kids that we need to really survey closely after this operation, because we know that they still need a lot of care.? We did help with small aspect of their symptoms, but we know they're going to continue to have issues. So that's also part of the research that we are doing, and as we get more patients that we take care of, that will allow us to do a better job of analyzing these patients, to also help with collaborating with centers like Johns Hopkins, All Children's in St Pete, and Boston, to work with them and with their experiences with some of these kids. So we can learn from them as well, and they can learn from our experience as to what certain things to avoid or certain things to do differently, and patient selection and all sorts of these things. So it's definitely a field that is still in some form of infancy, where we're still discovering things. We're going away from the dogma of they'll grow out of it, to a place where, no, we can do something about it, and then we're trying to figure out what's the best thing to do about it.

00:52:34

Dr. Krishna Pancham: Yeah, I think that's a very important question. Dr. Mokdad, as he mentioned, that this is a new surgery, and some of the things which we'll know in future, how the airways looks like after the tracheopexy. This is, again, a very unique aspect, where pulmonary is working closely with the surgery team, where we can do surveillance bronchoscopy six months, one year, even two years, to see how the airways looks like. And these patients, we have database collections, and we actively would like to research. And one important aspect of doing this program is to get some research funding. So we have applied for endowed chair, and hopefully that we will get it, and that will be a very unique aspect where we can give the data, not just for Cook Children's, for that matter, for the whole world, not just for the United States. Because this is a new surgery. This is a new treatment. Definitely it works. But what is the long term data? Everybody is always going to ask us that question, and we need those data so that we can deliver it to the pediatrician, to the family, and they deserve the answer. So far, we can tell you and tell our listeners that the surgery is very effective. We have minimal or no complications. And post operative surveillance bronchoscopy, which I have been doing, it has shown significant improvement in the airways dynamic as well as reduction in antibiotics, reduction in steroids, reduction in the ER visit, as well as hospitalization, ICU admission, and we have data for that. Hopefully we can publish that in future. But again, this is a very important question, which we need to answer, and hopefully we can with the funding.

00:54:21

Host: So as we close, what does the future look like for the field of pediatric, tracheomalacia medicine, what advancements are on the horizon, and most importantly, what does the future hold for these kids?

00:54:34

Dr. Ali Mokdad: I think the biggest change for these kids is, let's switch from you'll grow out of it to there's actually an option for you, and as I said, this is for the kids and for the families. From a advancement and what's at the frontier for this? I think a lot of it is learning about patient selection and learning about which patients will do good with this, which patients will do fantastic with this, which patients would require significant more attention, even after doing an operation. And by being multidisciplinary and looking at all aspects of this disease and the comorbid conditions that come with it, I think we set ourselves as Cook Children's and tracheomalacia program to be at the forefront of this, to be able to do the best we can for these patients.

00:55:27

Dr. Krishna Pancham: So I have a dream in 10 years. I feel that when I first came to Cook Children's, I felt that one day we will be a Center of Excellence for airway program, and that we will get reference not from just Texas, from whole United States, and hopefully we will be able to provide care for kids who have been struggling through many, many years and not getting the answer correctly or getting the cure correctly. So I feel that when we'll have a robust program, not just involving our surgery team, pulmonary team, but also involving ENT and GI team, as well as cardiothoracic surgery team, for in distant future, where we can work collaboratively in those patients care. Where we can provide them the comprehensive care and get reference and recognition for our wonderful program. We are very dedicated team and very enthusiastic team, where we can work together and build a very robust program at Cook Children's.

00:56:32

Host: So is there anything else we've missed that you'd like to add?

00:56:35

Dr. Ali Mokdad: As I said, the big part of this is, try to figure out which kid is not quite doing well, doing a good diagnostic bronchoscopy, and then knowing that there are options for these kids, I think the sort of the summary for this.

00:56:50

Dr. Krishna Pancham: Yeah, and my two cents is for the pediatrician or primary care doctor, and PCP is that If a patient is not following a usual trend, please think out of box and send them to us. We're happy to help those patients. Thank you.

00:57:10

Host: Fantastic. Thank you both so much for taking time from your busy schedules to share the exciting things happening in the tracheomalacia program and also for all you do for kids.

00:57:21

Dr. Ali Mokdad: Thank you very much. cx

00:57:23

Dr. Krishna Pancham: Thank you so much for inviting us.

00:57:25

Host: And for our listeners, find more information on the tracheomalacia program at Cook Children.org. You can also access clinical pathways on the Health Professionals section of the website, and while you're there, sign up for our Doc Talk newsletter. Want to hear more Doc Talk? Get our latest episodes delivered directly to your inbox when you subscribe to our Cook Children's Doc Talk podcast from your favorite podcast provider. And thank you for listening.

Tracheomalacia: Early Intervention Strategies for Lifelong Outcomes

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