Aerodigestive Management of Pediatric Aspiration - FULL SHOW

All right, well, good morning. Um, like Doctor Rutter said, we're excited to have you all with us, um, from across the globe, and we're gonna start by just trying to define pediatric aspiration, which is not really an incredibly easy task to do. So when we think about what aspiration is, there are a variety of definitions. Some would say that it's when any sort of solid or liquid matter passes below the vocal cords, and, and that's the end of the definition. Some would say that as it's really only truly aspiration, if you have things passing beyond the vocal cords, and you have pulmonary compromise, um, that goes along with that aspiration event. Um, one of the big things about aspiration is that it can be a, a silent process, where they, the child gives no indication that it's happening, or it can be a very obvious thing with lots of coughing, choking, and sputtering that goes along with it. So when we think about which children aspirate and which children we should be concerned about aspiration in, we break them into three main groups. Children who have an anatomic anomaly, so things like tracheoesophageal fistula, laryngeal cleft, children with a history of oesophageal atresia, or uh other reasons for esophageal strictures. We think about children who have any sort of neurologic disorders, so these are children who've got history of any sort of brain injury, cerebral palsy. And then we think of conditions that lead to significant peripheral neurologic compromise. So things like congenital infections, um, that can, can cause neurologic issues, uh, children who have esophageal dysmotility due to neuro underlying neurologic problems, or children with CHARGE syndrome who all have a very high prevalence of neurologic disorders. There's a group of children, and Doctor Rutter alluded to this in the introduction, that you should just assume it. Baseline that they aspirate until you have demonstrated that they do not. Children with CHARGE syndrome, 80 to 90% of them will have aspiration at some point in their lifetime. Children with OIT syndrome, any child that has severe neurologic compromise, regardless of the etiology of the compromise, and children with, again with congenital uh congenital infections such as varicella, coccack coxsackie, things along those lines. From the pulmonary point of view, you know, aspiration again is anything that goes below uh vocal cords in the lower airway that does not include gasses, no, um, in terms of what is the clinical significance of aspiration, the quantity of aspiration is a very significant factor, uh, so if aspiration. The event is a small, relatively small, isolated event. Most host defenses will render the event inconsequential by cough mucociliary transport. In the event of large or repeated aspiration events, then you have host defenses that will be overcome and inadequate. Most of us have had a single aspiration event happen to anybody, and the consequences will depend on circumstances, magnitude and the nature of the material. A single event of caustic substance aspiration can have lifelong consequences. So what we're here today is to discuss chronic aspiration, pulmonary chronic aspiration, and from my point of view, there will be repeated aspiration. And into the lower airways, so past vocal cords, but into the lower airways that do have effects of causing pulmonary injury or chronic respiratory disease, and the consequences of that chronic aspiration again will be determined by multiple factors the frequency and magnitude of aspiration, the nature of the material aspirated, not the same aspirating just small amount of water or saliva versus gastric reflux material. And the effectiveness of the host defense mechanisms and that includes the capacity to protect the airway and that has to do with sensation, consciousness, and anatomic or dynamic abnormalities, the capacity to clear material aspirated that has to do with the strength of their cough, respiratory muscle strength, whether or not they have restrictive lung disease, as well as other anatomic abnormalities, and last but not least, their immune response, whether it's. Exaggerated or absent, so in terms of who aspirates, so Dr. Hart already mentioned some of those for me there's 4 big groups. There's the premature babies, the ones with neurologic disabilities, those that have airway disease, either anatomic, dynamic, or functional disorders, and the gastrointestinal disorders. And then going between the anatomic and the functional, we already mentioned some of those, uh, so I'm not going to go through them again. And I would like to add a little bit more into some other syndromes that we encounter very frequently in our aerodigestive team. So of course the CHA already mentioned, but other patients that might have significant swallowing dysfunction include the Criducha, those that also have cranial nerve abnormalities like the CHAR but the Mobius syndrome, and anybody with significant craniofacial defects like the Pfeiffer, Croisson, and Trisher Collins. So the third part of the definition, so we all know that swallowing is a very complex process during which saliva, liquids, and foods are, uh, they, they go from the mouth into the stomach, uh, while the airway is kept protected. And the aspiration occurs when all this complex process fails, and then the saliva, liquids and foods can go into the airway. So swallowing is commonly divided into the following four phases the oral preparatory, the oral transit, the pharyngeal and esophageal phases. And we may have aspiration and complication in all these phases. So dysphasia can occur in one or more of the four phases of swallowing and can result in aspiration or the retrograted flow of food into the nasal cavity, and this is the definition of dysphagia and swallowing disorders of the American Speech, Language and Hearing Association. So the child who aspirates may present difficulties when feeding. Such as breathing difficulties when feeding that that might be signaled by increased respiratory rate, bradycardia or tachycardia, cyanosis, apnea, frequent stopping due to uncoordinated sex, swallow and breathe pattern, and desaturation, and also, uh, as we know, coughing and or choking during or after swallowing. Frequent congestion, congestion of the airway, mainly after meals, frequent respiratory illnesses, gagging, loss of food or liquid from the mouth when eating, noisy or the wet vocal quality during and after eating. They can take longer to finish meals. They can refuse foods or certain textures or types of food. They may take only small amounts of food into the mouth or on the opposite, overpacking the mouth or vomiting. And regarding the causes of dysphagia in children, we used to divide the causes into these three groups here. So we have the neurologic children who aspirate because of the incoordination of their swallowing process. Here we have the preemie babies, the children with cerebral palsy, the syndromic children, as Catherine and Cherie already mentioned, and the children with cardiac and pulmonary disease. The other big group here is the anatomic anomalies group where we We surgeons are very happy when we have this group here, so we have the laryngeal cleft and laryngotracheoesophageal fistulas, laryngomalacia, the children with glossoptosis, the Piaruba sequence, and also nasal diseases, mainly in small babies. And in the middle we may have this group here with mixed causes of dysphagia. Sometimes it's a child, we have a child with laryngomalacia who is also neurologic. So the underlying etiologies associated with pediatric feeding and swallowing disorders. Includes, as we already talked about, the complex medical conditions such as heart disease, pulmonary diseases, the children with some developmental disability, all the factors which can affect neuromuscular coordination can cause dysphagia such as prematurity. Low birth weight, hypotonia or hypertonia, also all the genetic syndromes. Next, the neurological disorders as we already talked about, and the structural anomalies such as craniofacial abnormalities, laryngomalacia, the fistulas, esophageal atrasia, kanal atrasia, and vocal fold paralysis. And also we can have dysphagia because of behavioral factors such as food refusal. And when should we suspect the child has an anatomic cause for the dysphagia? So all the children who present with associated obstructive symptoms of the airway, we should suspect they may have an anatomic cause. So children with snoring, retractions. Diagnosis and desaturation should undergo an endoscopy of the airway to look for this anatomic cause, but sometimes we have surprises, and children who present only with dysphagia as the main symptom may also present some anatomic cause for their dysphagia. Yeah just showing the retractions and this is a child, a syndromic child of stridor. So this child had a very bad dysphagia and also laryngomalacia. So we may perform a flexible fiber optic laryngoscopy here. We will talk a lot about this exam here in the next sessions. And also uh uh direct uh bronchoscopy, a rigid bronchoscopy to look for this uh anomalies. And again, this is defining aspiration because everyone looks at this slightly differently. So for the majority of the audience, food, drink, saliva, reflux, esophageal contents entering the airway, whether there is pulmonary compromise or otherwise. Now we're just. Um, so we're gonna move on and talk about what may be aspirated and how to diagnose it. And if we sort of think about the, the global overview of aspiration, You can aspirate food and drink. You can aspirate saliva, you can aspirate gastroesophageal reflux. Occasionally, you can aspirate esophageal contents. And so this is typically a child with an immotile or poorly motile esophagus, who's got a Nissan at the end of it and can just build up secretions in the esophagus, particularly overnight. And when they valsalva, some squirts into the stomach and some squirts up and can spill over into the airway. And what is aspirated influences both investigation and management. And the pretty picture is of an impedance probe. And so, this is a fees evaluation, a functional endoscopic evaluation of swallowing. And this would not be normal. This is showing a child with gross penetration, aspiration, not clearing the larynx. And, uh, and so what are the symptoms that we tend to see with aspiration? You may have coughing and choking while feeding. You may have a child with very wet sounding breathing, and so it can be. You know, Absolutely silent, where you have no idea that they're aspirating, aspirating based on clinical symptoms until you're seeing consequences. And the consequences can be acute life-threatening events, recurrent pneumonia. An insidious downward drift of baseline oxygen saturations. And so by that, I mean that one year, their baseline sats are 98, the next year they're 95, the next year, they're 92, then they're starting to need oxygen at night, they're needing oxygen when they're sick. So a progressive oxygen need. And eventually transitioning into frank bronchiectasis and progressive respiratory failure. And uncontrolled aspiration will have a marked impact on a child's quality and quantity of life. And so then you have to think about what are they aspirating? How do we test for it? Because not every child aspirates food and drink and saliva and reflux, and your management depends on what the child is aspirating. And so if we're thinking food and drink. You can think about a video swallow study, a functional endoscopic evaluation of swallowing. And these have some limitations. This is a window in time, and these tests are often non-physiological. Doing a fees evaluation with a telescope shoved down a child's nose and you go and throw something in their mouth and see what happens, isn't truly physiological. It can be very useful. Uh, the video shows a child with very bad laryngeal candidiasis, who's got stridor and aspiration and was going to have a tracheotomy. The fees evaluation showed the candidiasis, got treated, needed no other intervention. And then in terms of interventions for food and drink, you can limit oral intake and thin fluids drunk fast to the highest risk of aspiration. Or you could put in a, you know, bypass the pharynx as a route towards the stomach, so a gastrostomy tube, uh, or some variation on that theme. And an important piece too, Mike, just for our audience members to hear too is we often will recommend, Getting both of the video swallow study and the fees because they are complementary tests you can see different things and also different parts of the swallow, so it's often important that you may end up getting both and it's important to explain that to your families because they may think it's the same test, correct. And so, uh, we've gone from food and drink to saliva. And again, this is a child who's had a laryngeal cleft chronically for a very long time, chronic salivary aspiration, and every time we did a bronchoscopy, he appeared to have the same oyster coming out of his right main bronchus. Um, and so what tests can we do for saliva? We can do a radionucleotide spit scan where we put a drop of radioactive. Uh, tracer on the tongue and see if it goes to the stomach or the lungs. It involves a little bit of radiation. Again, it's a one-off test. It's a window in time. In terms of interventions, you can try something to reduce the amount of saliva you make, like glycopyrolate, usually not very effective. You could temporarily paralyze the major salivary glands with Botox. You could do a drool procedure, and we'll talk about that a little bit later. With gastroesophageal reflux disease. Again, is this. Esophageal reflux or extraoesophageal reflux or extra oesophageal reflux causing aspiration, which are all different things, and the tests we can use, an impedance probe is clearly the best but not necessarily widely available, and uh, You can do a barium swallow as you can see on the right. Again, a window in time, and just because you reflux does not mean you necessarily aspirate, and the typical interventions are, Gastro jejunal feeding or a fundoplication, and medication, generally speaking, stops acid but does not stop reflux. And then we've got some other tests, CT scanning and Cherie will be talking a little bit about that later on, flexible bronchoscopy and microlaryngoscopy and bronchoscopy. And so, uh, rigid endoscopy is very good at looking at anatomical problems and, uh, whereas flexible bronchoscopy, bronchoalveolar lavage can be very useful at looking for signs of chronic aspiration. CT scanning Excellent for evaluate evaluating long-term consequences, but you're looking for damage already done. It tells you about the past, not necessarily about the present, and it generally requires an anesthetic, radiation, and a degree of expertise from your radiology team. Flexible bronchoscopy. I'm not gonna dwell on this because Cherie's going to. Rigid bronchoscopy. Again, this is excellent for looking for anatomical anomalies, great optic optics, but it's a straight line skill. And some things barn door, laryngeal cleft, tracheoesophageal fistula. And so I'm gonna do a very brief case presentation because I think I'll, where possible, individual cases are the most useful in terms of, uh, trying to understand how we actually manage an individual child with aspiration. So this is a little girl, she's got Down syndrome. She's 5 years old. She's had an AV canal repair, and it was a little problematic. She had a couple of cardiac arrests with a bit of a cerebral injury. And ended up with a tracheotomy, a vent, and a subglottic stenosis, as you can see in this video. She's had pulmonary hypertension, which is resolved. She's got an impressive silicone allergy, and she has had some recurrent respiratory illnesses. And Because She was doing well clinically. We reconstructed her airway. And this is 3 months after a laryngotracheal reconstruction. And she was successfully capping her airway at this point in time. So she's got a small trach tube, she's got a cap on it. She's got a little bit of suprastoal collapse, but she's actually capping reasonably well. However, She developed an increasing oxygen need. And had a CT scan. Consistent with bronchiectasis and a previous CT scan two years earlier had not looked like this. And so in June this year, She had a bronchoalveolar lavage which showed 6% lipid laden macrophages. And as a comparison, we'd done previous bronchoalveolar lavages that had shown an improvement with time. To the point that we were willing to reconstruct her airway. So the question is, did our anterior posterior graft predispose her to aspiration? Her video swallow study looked pretty good. Her esophagogastroduodenoscopy looked normal. Her impedance probe showed that she refluxed. And so a question to the audience. Do you think this girl is aspirating? This is easy. This is yes or no. OK, well, that's pretty comprehensive. At the moment we're running 100%. She's aspirating. It is a pretty compelling story. And so what other tests could we do? And so this is one of the things we chose to do. We dye tested her. So what does that mean? That means that we can put a drop of green food dye in anything she's eating or drinking and see if it comes out of her trach tube. We can put a drop of green food dye on her tongue, which is a test for salivary aspiration. We can put a drop of green food dye in her G tube feeds and see if you suck green out later. This is something you can do repeatedly. You could do it 3 times a day. You can do it for 10 days in a row. So it's fairly physiological, and green is not a natural color. So if you're sucking it out of the tracheotomy tube, you've got a pretty good idea that you've got a problem. And so this is not a window in time. And it's very cheap, it's very convenient, and it's got good customer buy-in because it can really show someone if they are aspirating. And so what did we find with this girl? The dye test was negative, interestingly, and, uh, to motivate her, her older brother also dye tested himself. He doesn't have a trach tube, so he just got the green tongue. And, uh, we found that she didn't aspirate saliva, she didn't aspirate food and drink. One of the questions we're still wondering is whether she might be aspirating reflux at night, and we're still trying to resolve that. And so what are some of the advantages of, Food and drink and die testing so. You can test different consistencies of food and different positions of the child. And so some children we've seen are more at risk of aspirating particular consistencies, thin fluids drunk fast being the classic. We can put a drop of dye on the tongue, so even if the child is not taking any food or drink, it's still a valuable way in a child with a tracheotomy who doesn't have a grade 4 subglottic stenosis of seeing whether they aspirate. We can put it in their GTube feeds, and this is a fees evaluation of someone having a reflux event, which you'll see fairly shortly. And, uh, again, do it at different times of day, class, and there goes the reflux event. That was disgusting. Um, and so this is something that you can potentially do at night to see if they're having a problem at night. And again, this has shown particular groups are at risk. We talked about this before. The limitations, it's only useful in patients who've got a tracheotomy and don't have a grade 4 subglottic stenosis. There's been some concerns about whether you could actually have met hemoglobinemia with methylene blue. And this is really not relevant in this population with green food dye. And so. Firstly, we need to diagnose what is being aspirated. If there's an anatomical problem, correct it and. With each substance that they aspirate, there are potentially ways of investigating it. And dealing with it. So our uh protocol of the diagnosis of aspiration, uh, begins with a non-instrumental assessment, uh, generally with a, a speech language pathologist with us in the clinic. Um, and this is a very important, uh, evaluation because we have to first of all if this child has an adequate jaw movement, a tongue movement, uh, to check the child's ability to compression and suction and the infant's ability to maintain, uh, the physiological state during feeding. And after that, next please, after the non-instrumental assessment, we can go on and proceed to an instrumental evaluation. Next, please. So the instrumental evaluation, as Mike already talked about, um, is conducted to to diagnose the aspiration and to determine the nature of the swallowing disorder. We have mainly these two instrumental evaluations here, the VFSS and the FIS, and I'm going to talk very briefly about them again. So next. So VFSS is still the gold standard method for the diagnosis of aspiration. It evaluates all the phases of swallowing, but we have some problems because it needs contrast to do the test. It uses radiation. That is a very big discussion nowadays about the radiation in children. And the patient needs to go to the radiology unit. It cannot be performed at bedside. Next, next slide, please, uh, the fees evaluation, we have an endoscopic view of the swallowing at the same time we can diagnose anatomic anatomical alterations and anomalies. It evaluates directly the pharyngeal phases of swallowing and indirectly the all all the other phases of swallowing. It's a non-invasive, relatively cheap and dynamic study, and it permits serial examinations. We can do it every day in a child if you want, because it doesn't use radiation. It evaluates also the laryngeal sensibility and saliva aspiration, that's very important, and it can be performed in any setting, so ICU at the clinic, bedside, everywhere, so it's really very comfortable. to do and as Sandra already mentioned, it is actually these two exams are complementary. They are not one doesn't substitute the other, so we may have to do both to have a very good to have a good diagnosis of the problem. The flexible airway endoscopy that we're going to talk later. And the next place, and the rigid bronchoscopy as well, and this is a video just showing you, uh, this was a very easy child to perform the feast, but it's not always like this, so you can just run the video, please. The bone. Fun yeah. Thank God ain't no like this, hey. It's to the best of you know and not the commendochazulu gree ki ed me. What if this woman's gonna show you. Wisdom. Fine, it's just to show you that this child was really very easy to, to perform the exam, but it's not always like this, so we may try, but it's not always so easy to, to do this test. All right, diagnostic, differential diagnosis of aspiration. OK, so I'm gonna talk to you a little bit about differential diagnosis of aspiration. And there's a significant challenges in the diagnosis of aspiration and when we differentiate from other uh similar uh clinical presentations. One of it is the episodic nature of aspiration. Not all kids uh aspirate every day. The other one is that there's different thresholds for disease depending on comorbidities, uh, you know, the sequela of aspiration will depend on the capacity of the patient to clear and other things, as well as other overlapping symptoms caused by comorbidities like asthma, chronic lung disease of prematurity, um, and the fact that we do not have a specific gold standard diagnostic test to tell me whether or not, uh, respiratory illness or clinical presentation is actually due to an event of aspiration. When we talk about differential diagnosis, uh, one of the most common things, especially when we talk about the general population with not many other comorbidities, is the fact that children get sick, and the more sick they get and when they have been diagnosed with pneumonia, even when the clinical symptoms do not support it, then the next time they get sick, it's actually more likely that they will again be diagnosed with the pneumonia, almost as a self-fulfilling prophecy. And that's what we call the daycare center syndrome, where children are thought to have more of a significant ongoing recurrent respiratory problem when in fact it's nothing but clinical exposure to other children and frequent respiratory infections. So starting with the most common conditions that might be confused in terms of chronic aspiration, we have asthma, of course, where the history typically includes patients with chronic rhinitis, conjunctivitis. Uh, they also might come with a history of recurrent pneumonias that do not typically. Present with the typical fever, uh, oxygen requirement. Presentation tends to have a seasonal quality to it, and there's also some identified environmental triggers, changes in weather, uh, or exposure to things that give them allergies. In terms of the imaging, as we know, plain films are not really very uh specific or sensitive to differentiate between the different possible causes of respiratory infections or respiratory problems, uh, but in asthma, you will typically see hyperinflation, increased perihilar markings, and peribronchial thickening, all of which you can also see in patients with chronic aspiration. The second less common one, but one that needs to be in your differential will be cystic fibrosis. These are patients that do not have the typical seasonality or seasonal presentation like the asthma kids, but do have this chronic productive cough, history of. pneumonias, but they usually have other more systemic conditions, typically failure to thrive, intestinal malabsorption, as well as, uh, they tend to grow organisms that are uncommon or atypical, like Pseudomonas. In terms of imaging, uh, differentiation, well, again, there's findings on cystic fibrosis that can be very similar to findings on chronic aspiration. Bronchiectasis will be one of those, uh, clinical presentations. However, the distribution of bronchiectasis in patients with cystic fibrosis will not have the typical dependent areas distribution that you see in patients with chronic aspiration. But you can have the treating bod opacities, you can have multifocal air trapping due to chronic inflammation, as well as bronchial wall thickening. And in this case, all of these findings can be seen in patients with chronic aspiration, but it's more related to the location of these changes that will point more toward the likelihood of it being chronic aspiration. The third, uh, rare, but still, uh, similar in presentation will be primary ciliary dyskinesia. And these again will present with chronic respiratory symptoms with wet cough, recurrent pneumonias, uh, and will also typically present with recurrent upper airway infections like purulent rhinitis, otitis media. In a subgroup of these patients, you will have situs inversus, which is about 50% of those with ciliary dyskinesia. And then some of them will present with the classic Carthaginner syndrome that includes the cytosin versus, the sinusitis, and the bronchiectasis. Diagnosis at this moment is recommended to be done with a combination of ciliary biopsy, looking for ultrastructural celiac defects in electron microscopy, as well as genetic testing. Then we have patients with immunodeficiency who can also present with recurrent infections, many of them primarily presenting with respiratory infections. However, GI and skin infections are also very common in this population, and these patients also present with a typical pathogens that is not the classic presentation in the early presentation of chronic aspiration. They will have systemic uh problems like failure to thrive, and they also might have complications when given live vaccinations, as well as other things like chronic diarrhea, skin lesions, and a family history of immunodeficiency. Now, let's talk about diagnostic evaluation, uh, for the purpose of trying to differentiate between these, uh, Conditions. We already talked a little bit about imaging, and we know that plain films like chest X-rays are really non-specific. However, you will be looking for things that are associated with chronic inflammation and mucous plugging, uh, that will actually lead to hyperinflation of areas of, uh, unequal hyperinflation. Uh, subsegmental infiltrates, peribronchial thickening, uh, and in patients with more, uh, chronic disease where they have already developed significant bronchiectasis, you can have in chest X-ray what we call the tram tracks, which is essentially a linear, uh, image that happens alongside the bronchi that essentially delineates the affected, uh, dilated bronchi. Uh, in addition, you are always looking for dependent areas changes. In babies that are still drinking from the bottle, that typically take the bottle in a supine position or semi-seated position, you will see more commonly the posterior upper lobes, especially right upper lobe affectation. However, children that are toddler years or already sitting upright, then you will look for changes in the basal, um. areas of the lungs. However, there's a significant number of our patients that aspirate during sleep. So, that will cause some changes in the posterior dependent areas of the lungs as well as the lower lobes. And it will be interesting that some of them might have asymmetric um changes, one lung more affected than the other one. So, part of your assessment needs to include what are preferred positions to sleep, uh, or how does the baby feed, especially those that are breastfed. Sometimes they prefer one breast versus the other one. So then they will have more changes of aspiration. Depending what is the dependent side when they're feeding. Then we have the CT scan, and we said this is the much more sensitive study to look for changes that are commonly seen in chronic aspiration. And these, uh, again, we're looking for the distribution. It will initially affect mostly the small airways, so in which case you will not see the gross changes of bronchiectasis, but you will see air trapping, subsegmental air trapping. You will see areas of consolidation or electasis, and you can see the treating body opacities, especially in children that are less than one year old, who have chronic aspiration. Bronchiectasis tends to be obvious in patients with very severe aspiration even before 12 months of life, but those that have more intermittent events of aspiration, it's rarely that you will see significant changes less than a year old. Therefore, uh, for most of our infants, we do not necessarily get a CT scan unless they have, uh, gross changes on their chest X-ray and their airway evaluation. Again, bronchiectasis, which is one of the landmarks in imaging studies that suggests chronic aspiration, um, is very high prevalence in patients that have sustained chronic aspiration, can actually develop a very young age, uh, and the clinical history and physical examination are not useful predictors to determine if bronchiectasis is already developed. Um. The long term consequences may be prevented if this is diagnosed early on and appropriate measures are taken to control the ongoing aspiration. So, now we talk about the diagnosis of swallow. So, when we're talking about diagnosis of swallow, we, of course, are looking for uh GI disorders that might be associated with aspiration. Uh, and studies that can uh be useful will be um EGD, upper GI and small bowel follow-through, impedance probe, as well as a swallow study with a video fluoroscopic study. Uh, you're looking for GI problems like dysmotility, sphincter dysfunction, of course, gastroesophageal reflux, and how that reflux correlates with respiratory symptoms and that we can obtain through an impedance probe. And you're also looking for other anatomic abnormalities that might be associated with chronic aspiration, including uh tetralogia follow-up, as well as uh other anatomic or motility abnormalities of the upper, um. Gastrointestinal tract. Then a re-evaluation. So, I will move through this uh relatively quickly because this was discussed in more detail in other parts of our, uh, cast today. Uh, but essentially, we have two, big exams. We have the, uh, microlaryngoscopy and bronchoscopy, and then we have the flexible bronchoscopy, no? And they are, uh, studies that are done concomitantly because each one will give you different information. So the MLMB, microlaryngoscopy and bronchoscopy, as you all know, evaluates the anatomy, uh, including looking for laryngoesophageal cleft, glottic webs, uh, as well as scarring of the glottis, also glottic area that might compromise, uh, airway protection and vocal cord movement, um, or, uh, resulting in tetering of the vocal cords, true or false, and erythenoids, as well as circuesophageal fistulas. The flexible bronchoscopy essentially looks for not only the anatomic abnormalities, uh, but also for, uh, dynamic abnormalities that might be contributing to swallowing dysfunction, uh, including the upper airway, but we're also looking for other markers that support the diagnosis of aspiration. Uh, we are looking for conditions or comorbidities that might explain the chronic symptoms, OK? Cause as we said, part of your diagnosis. Uh, that include tracheal bronchoalacia, vascular compression, as well as airway abnormalities, anatomic dynamic that we just mentioned. These morbidities by themselves could be mimicking the symptoms of chronic aspiration. However, at the same time, if these are comorbidities to chronic aspiration, this will be uh contributing to the sequela of aspiration as it might limit mucus clearance as well as appropriate ventilation that allows for better clearance. OK. So, as part of our flexible bronchoscopy evaluation, we perform a bronchial bio lavash. Essentially looking for things that do not belong to the lungs. We are, uh, identifying infection, trying to characterize the cellularity of the lower airways, as well as looking for blood or lipid uh into the macrophages, being lipid-laden macrophages, the most commonly used aspiration biomarker. The indirect chronic aspiration markers will include elevated number of lipid ladder macrophages, which if you're measuring the index will be typically at least more than 90. If we're measuring the percent of lipid ladder macrophages, we use 20% as our cutoff. In terms of neutrophils, which is a marker of inflammation, normal would be to have 5% of cells to be neutrophils. Anything above that, and especially anything above 40 or 50% of neutrophils suggests uh inflammation. We also look for presence of upper airway squamous epithelial cells, which suggests chronic aspiration of oral secretions, and then we look for the presence of a large number of oropharyngeal bacteria. In this case, talking about more than 100,000 units per high power field will be considered a positive culture. Going back to lipid ladder macrophages, which is right now kind of the number one, marker, some places, as I said, measure the index, which is essentially, uh, quantifying 100 cells and characterizing them from a score of 0 to 4 and then adding up the score. Uh, there are problems with regards to what is considered a threshold of significance of that index. Some people take 90, some people take 100, some people take 150. As a general rule, more than 90, an index of 90 suggests, uh, aspiration. So, where does the lipid come from? Is, uh, an elevated lipid ladder macrophages, uh, Patho pneumonic of aspiration, not necessarily. You can have uh elevated lipidator macrophages due to just uh natural debris in the airways, including dead cells such as neutrophils and other macrophages, which are typically increased status post infection or due to any reason of causing chronic inflammation, and it can also be there due to circulation as in patients status post bleeding or airway surgery. So, lipilar macrophages, even though it's the number one marker used in this moment in time for diagnosis of chronic ongoing aspiration, is a limited tool. Why? Because there's a variable amount of lipid in any aspirated material. If the child is not fed orally or is not having reflux aspiration when fed per G tube, then you might only have saliva aspiration which does not contain lipid. Uh, there's variable time between the aspiration event and the sampling of the BAL, and that will have an effect on the metabolism of the lipids and how you find them, uh, in the macrophages, um, as well as every person have a variable rate of lipid catabolism. There, um, we've actually had a question come through. Could you comment on the influence of the presence of a tracheostomy tube on swallowing function and aspiration, and a follow up to that, should you maintain a tracheostomy tube for the single reason of aspiration, or would decanulation improve swallowing? And so I'll ask the panel. I've got some opinions, but let's, let's go and sort of while we deal with the minor technical issue, talk about whether a trach tube actually contributes to aspiration. So Catherine, you're looking really motivated to answer that. Cherie looked like she was ready to chomp at that bit. Um, so we know that having a trach tube in. Place changes the dynamics of laryngeal elevation, uh, but most of the studies have shown that it doesn't create aspiration and, and I'll let Cherie weigh in as well, but if you have a child as to whether or not you should take a trach out in order to treat aspiration, I would say that would be a very, very rare circumstance that that's going to be the solution. Now, just because a child aspirates, doesn't mean you have to keep the trach tube for that sole indication. It depends entirely on the degree of aspiration and whether or not you feel the child is going to be able to adequately protect their lungs, uh, in the, in the presence of that aspiration. Totally agree. I think that one of the things that I want to reiterate and from the pulmonary point of view, that's why we work so good together, is that the plan for the cannulation needs to take into account not only the patient's capacity to ventilate, but the patient's capacity to clear their airway because the tracheostomy definitely facilitates airway clearance, so. It's not what you get in, it's how you get it out. So in that regard, I will strongly recommend against the cannulation until we have proven that this child by having a probably prolonged uh capping trial can go through illnesses and has regular advancing oral feeds without having to use a tracheostomy tube to help clearing so tracheostomy. In my, from my point of view, it's actually an adjuvant into the management of aspiration and keeping the lower airways clean of aspirated material. Any views on, say, speaking valves, passing mu valves, or the equivalent? Oh yes, and we're actually going to talk about it a little bit later too, but I, there is actually no significant evidence to say that the speaking valve keep you from aspirating. However, it is my experience. That the speaking valve by allowing for glottic closure and generating a better cough is actually going to help you clear secretions aspirated from my point of view, um, it will also help to create a little bit of end expiratory pressure that distends the airways and help on clearance. So even if there's no evidence that it will decrease aspiration coming in, it will actually help with airway clearance and decrease the sequela of aspiration. And so as a summary. It's best to leave a child with a small trach tube plugged for a while before decannulating if you're concerned about aspiration and don't decanulate them unless you're confident that the aspiration can be managed. Reasonable comment? Absolutely, yes, I agree. So I'm going to make a small comment that we probably didn't touch upon that's relative to one of those questions about tracheostomy tube placement. And aspiration. Probably a perfect example are CHARGE patients, which frequently at a young age, they may need a tracheostomy or other interventions to manage their aspiration of salivary secretions, and you will often be quite surprised how well they do over time as they develop. And there's this element of developing compensatory strategies that you can often deannulate those kids, um, because they have improvement in their management of secretions and aspiration. And one more thing in that regard, I think that the, uh, you know, being a pulmonologist, we all know that the lungs is the most important part of our body um and in that regard I think that I see my function, especially in the aero digestive team as trying to protect these kids from developing long term sequela that might not be uh. You know that you cannot resolve, it comes to a point of no return as we manage, you know, some anatomic abnormalities, surgical or non-surgical dysfunction, uh, and sometimes it's just a matter of maturity, so I will do and we will talk about management, medical management. Whatever I need to do to keep the lungs healthy, preventing sequela as we either wait for the kid to mature, uh, you know, oral intake, uh, function or we take care of the other anatomic abnormalities that are, um, factors contributing to the aspiration. Uh, might there be a continuum from normal or average to abnormal? In other words, what percentage of normal children have any degree of aspiration that might be recurrent, but not significant in terms of pulmonary complications. And so this is really all of us aspirate occasionally, at what point is it abnormal? And so, uh, I think I'll let Cherie tackle that. OK, so absolutely I think that everybody, um, has aspiration and children as they are advancing into their natural bottle to pures to regular feed will have some events of aspiration as they, you know, get used to in new textures, uh, and new volumes. Uh, however, to me again. Aspiration becomes clinically significant when you have significant soiling of your lower airways and with that the sequela with chronic respiratory symptoms as well as recurrent respiratory infections and more advanced to it of course the development of pulmonary injury, so in terms of normal, so again, the frequency and magnitude of aspiration and how it interferes with normal feeding, weight gain, uh, and. Uh, whether or not the patient is out of the normal expected range of number of infections and complicated, uh, respiratory symptoms will kind of tell you where does this child fall within the acceptable normal category and the ones that don't, uh, to me I see aspiration dysfunction just like speech delayed in a way like there's a lot of kids that learn to speak a little bit later. Some of them might need intervention. Some of them just get it, uh, and start on a little bit later and then just do very well on aspiration we do have a relatively large group of patients that will have some respiratory symptoms with oral intake, uh, and then might actually have some more respiratory symptoms when challenged with like increased upper secretions with illnesses and things like that, but they do not necessarily develop the sequela or the consequence. What is the percent of those? I honestly do not know. We have the bias of being a supra tertiary center of referral, so most of the patients we see are the ones that fall into the pathologic category, but having practiced general pediatrics for 10 years before doing pulmonary, I can tell you that it's actually not an uncommon history of the patient having mini choking events when they have a cold that do not necessarily represent pathology. A few people asked about foreign body aspiration, particularly if you've had a section of lung completely occluded by a chronic foreign body of long standing. And so a couple of people have asked, what do you do if you've got a foreign body that's say, been in a subsegmental bronchuss for 3 years? And so, and that, that's a relevant question. It's, uh, It's not the primary aim of this global cast, but I think it's something of interest to a great deal of the audience. And I think the easy answer is, if you can get to it, take it out. And then it's a question of, is the lung terminally damaged or is it resurrectible? And so I think the answer to that is a case by case basis, because we've seen some children who have had a segment of lung that has not been functional for a long time, and then has been reconnected and has actually become functional again. The time period before you completely lose the lobe is variable. And so I think it's a minority of patients who after a year, can actually regain functional lung tissue, but definitely some of them can. And then it's a question of what are the consequences. And sometimes a lobe will basically involute. It's almost disappeared, and you've really not got a great deal of consequences thereof. Um, sometimes you'll end up with a segment of chronically damaged lung and bronchiectasis where you're having to consider doing a lobectomy. And we are going to move straight into the medical management of functional aspiration. And so Cherie's gonna lead that discussion, as you can see, she's been joined by Aaron Garrison, one of our pediatric surgeons, who he's looking particularly photogenic today. And so, uh, Cherie. OK, so, um, essentially, you know, uh, in general functional aspiration means that there is no anatomical abnormality or defect causing the aspiration or contributing to it, so the number one, group of patients that will present with this, uh, dysfunction will be those with either central or peripheral neurologic problems, uh. We're gonna be talking, we're talking about medical management of things like xyorrhea, uh, airway clearance limitations, and then the surgical aspect of the functional aspiration will be dual procedures, for example, G-tube, Nisscent from the application, tracheostomy, uh, for improvement of clearance, etc. etc. So from my point of view, medical management should target 5 different aspects of the, uh, physiopathology. No, Number 1, of course you need to decrease aspiration if in any way possible. Rarely ever we get a complete zero aspiration in our management. Number 2. Improve airway clearance number 3 address the quality of the aspirated material and we'll talk a little bit more about it in a minute and control inflammation as well as treat or prevent recurrent uh infections. Those are the five aspects that I uh managed directly from my end. So going to number one, we're talking about the uh decreased events of aspiration we already discussed diet modification so you see what are the things that they can eat safely in terms of syorrhea or saliva aspiration will be Botox or anticholinergics. Other things that we sometimes forget is like some children that has borderline swallowing dysfunction. It's not on. Until you challenge them with illnesses or allergies, um, where we have significant increased load of oper secretions that's when they aspirate so you need to be on top of controlling rhinitis and sinusitis um and then in patients with tracheostomy one way to decrease events of aspiration sometimes might be the initiation of positive pressure ventilation. In terms of aspiration of reflux material from medical management will be a reflux precautions and that includes especially in children uh looking into the volume, the timing, the rate and the quality of the feeds when we talk about quality of the feed. Sometimes we forget that some children that have GI dysfunction with this motility and delayed gastric emptying, you need to think about those things when you're selecting the formula or the way you're feeding them. The longer it takes for them to empty their stomach, the more likely they'll aspirate. Then other things, uh, regarding whether you do G tube or J feeds, and that depends on the pathology of the child. Airway clearance. So if I cannot keep them from aspirating even intermittently, then I need to improve whatever gets down there to be clear. So first we need to be aware that there are certain children that have comorbidity in airway clearance tracheal bronchoalacia will be one. Patients with airway compression, stenosis, or hypoplasia of the airways will have more difficulty clearing, as well as those with restrictive lung disease, may that be congenital or acquired. Congenital, well, the neuromuscular kids hypotonia or chest wall deformities and again acquired will be children with thoracotomy or scoliosis can develop issues with airway clearance. The cough and their capacity to clear is compromised and those that has had, uh, esophageal or cardiac surgery, you need to always remember about the possibility of vocal cord dysfunction and diaphragmatic dysfunction or paralysis. So look for those in terms of airway clearance, many modalities, it kind of depends on whether the patient can cooperate with the therapy or not, uh, extra pulmonary precaution in a non-cooperative patient, intrapulmonary precaution like a cappella aerobica in someone that can cooperate during the, uh, therapy, and then we use munculitis cough assist, and we already discussed about how the speaking valve could actually help with airway clearance by improving your capacity to cough or stenting the airways for better air movement. The third one would be, uh, sorry, we have technical difficulties again so quality of aspirated material and that really targets more of the we need to control the bacterial load of the aspirated material so don't forget that tooth decay in some patients, especially higher risk in patients where you control saliva. One of the secondary side effects is that you might be at higher risk of developing tooth decay, so these kids need to be followed closely for, uh, you know, having oral hygiene and then antacid medications that we'll probably discuss further on but it's less irritative to have non acid reflux aspiration than that that is highly acidic. 4 would be decreased inflammation. I personally think that a great part of the sequela caused by aspiration sometimes it's more related to the chronic inflammatory process than the repeated infections themselves, so I typically use a combination of inhaled steroids and systemic anti-inflammatory medication. Not systemic steroids as first line of chronic management but as management for acute events of known aspiration to rescue them from uh expected pneumonitis and then the azithromycin, which idea we take from our chronic uh lung disease patients with cystic fibrosis or primary celiar dyskinesia that pretty much help, uh, a great number of our patients to control, uh, the severity and the frequency of exacerbations. Can you go to the next one? So, and then last but not least is infections, and this is a little controversial because the question is, OK, so who needs uh antibiotics? What antibiotics do I give? If they have a trach tube. We always try to get cultures and sensitivities. These patients with recurrent infections more likely to develop resistant organisms, so you need to be looking for those. The other thing would be whether or not they have developed already bronchiectasis. My patients with bronchiectasis, I think of them as patients with cavities that have more difficult clearance. Therefore, antibiotic course should be in a longer, so 10 to 14 days instead of 7 to 10 days. And then the question, uh, who do I treat with prophylactic antibiotic, and that will be more likely cycle inherent antibiotics like Tobramycin or cholistimate, uh, and those will be those that fall into the very severe category with significant development of bronchiectasis and pulmonary injury or those that have significant frequency of infections, uh, where you will use them either every other month or 14 days of every month. We're gonna go to the next audience participation question. I've already got a a a. A really quite impressive look from Cherie just then, and that's because we very deliberately put in option B, which is really based around the, you shouldn't use a Paymir valve when you're asleep, which is why I put that in there. And clearly a lot of people do, which is fascinating. I'm not convinced a cuff tube really helps aspiration very much. And so let's firstly, Hugo, Claudia, any comments about whether you think cuff tubes are very useful in terms of actually preventing aspiration, Claudia? Yeah, well, they can minimize aspiration but not actually stop aspiration because we cannot inflate the cuff too much in order not to cause any damage to the mucosa. So I don't think it will stop aspirations. This is not correct. By experience and we already talked about the passing your valve, so it does not decrease aspiration, it improves, uh, clearance, uh, definitely I will not rely on the cough just like Claudia said because the injury or dilatation of the trachea by inflating it to the point that you decrease the leak enough to actually have an effect on aspiration is not acceptable, so that's not, uh, to be done. And then in terms of positive pressure I think that's the right answer we do have patients that do not necessarily need positive pressure for gas exchange or ventilation, but they do benefit especially during sleep, those that have reflux aspiration from positive pressure either a CPAP or BiPAP uh to. Increase, uh, the events of aspiration, especially during sleep. I think the key with any of those things though is that none of them prevent aspiration. They're all methods that you can use to help manage aspiration. And that's the big thing to, to keep in mind. These are all tools we can use to prevent those pulmonary and respiratory sequelae of aspiration, but they don't prevent the aspiration. Would you ever consider using a Paymir valve when a child is sleeping? Absolutely not, no, I agree. Why not? Well, as we know, Passimirba allows ventilation through the tracheostomy on on inspiration, but does not allow exhalation through the tracheostomy tube. And uh the reality is that when a child is asleep and uh there is a little bit more relaxation actually you can have more issues with mucus clearance limitation or things that accumulate around the trach tube so your exhalation can be compromised with obstruction. And then you have a patient that essentially can inhale but cannot exhale, so no, the other reason is, uh, passing valves are well known to cause over dryness of secretions so mucus clogging is something that is more likely to happen when you have a passing valve, especially for prolonged periods of time. So no, no passing your bath during sleep. Great to be here speaking to everybody out there, uh, in the global class community. So, when we think about surgical management for functional anomalies, sometimes it's thinking about where it's coming from, uh, and where is the main source of what you're trying to deal with. So if it's PO intake, maybe it's actually avoiding PO intake. So that means something like a G tube. If it's saliva, it may be looking at something like a drool procedure or a laryngotracheal separation. Uh, with reflux, it can be actually moving to several different procedures such as, you know, looking at a like a bypass where you either like go to a J tube feedings, you look at niss and fundoplication, you look at is there some anatomic component, like is there, um, a stricture that may be leading to more issues with a potential reflux and aspiration. So you look at repairing those, you can look at doing a sphincter myotomy or ruin Y. So again, planning, it's all based on what's being aspirated and then when is it being aspirated. So then all of those interventions and investigations that you do beforehand are actually what inform you on how to move forward. So for instance, when you're thinking about is it nocturnal, is it daytime, um, is it esophageal contents, like those are the places where it can be especially challenging and the places where again that team management can play a key role in making decisions about what your intervention is for aspiration. So, uh, I'm going to touch a little bit on, on one of the areas that we often see and how we deal with it. So unilateral vocal fold paralysis, for children that have it and then have issues not just with voice, but with, with aspiration as well. If you look at globally for unilateral vocal fold paralysis, some of these kids can be observed and just, you can wait it out to see if they get recovery of function depending on how the injury occurred to begin with. And then it's looking at the different interventions specifically to address the paralysis. So that can be doing a laryngoplasty of some sort, whether it's the more formal piece with cortex or silicone versus an injection and arritinoid procedures. And then lastly, one that we'll touch upon is relatively newer in the last few years in terms of its popularity in children is looking at a reinnervation procedure. So temporary laryngeal injections are really nice as a test drive procedure. Um, it does have to be done in the operating room. Typically, we will do an overnight observation, um, with a child that gets it for the first time. You can repeat it before doing a more permanent operation, and we tend to think of it as a bridge between those more permanent operations. So what are some of the early injection benefits? So this is when you know there's been an injury to one of the recurrent nerves. You identify the vocal fold paralysis, and it's very early on. So I'm talking in the 1st 1 to 3 months. So there's several studies in the adult literature that may suggest that this may lead to less need for doing more permanent procedures. Uh so we always kind of think of it as the rule of thirds. So 1/3 will get better, 1/3 will be paralyzed, but in a good position for both voicing and swallowing. And a third will be in a poor position where you're going to look at doing something more, really not a lot of um research that's been done in children. Interestingly, there's some work that's been done looking specifically in the cardiac patient population, uh, in terms of improving aspiration with injections early on. Uh, and so I think this is an area for that's in need of more research and certainly a place where probably you need to look at doing collaborative work with multiple institutions. So how do you choose your material? So, um, this is a question we often get because there's a, a plethora of different things you can inject into a vocal folds, anything from gel foam to the radius prolein um, materials, Restylin, Symetra, and fat. And so a lot of the choice of what you use is influenced by the time of how long you want that to last. The material properties of what you're using, so it doesn't really work. The risk benefit profile of that particular material, it's ease of use, it's availability in your particular, whether it's institution or country, and then you add in the cost as well, um, and so some. Nice studies actually done in dogs um by Dr. Zeitel's group looking at how these different materials sit in a dog larynges and how long they last. And interestingly, um, these, the radius ones don't tend to actually work that well, so the gels tend to dissipate very quickly in the, in the dog animal. So within a couple of weeks, they're basically gone. They don't really achieve what you really think they are doing. Um, I don't really like the more permanent radius because I've had to take it out of a few people and when it gets put in the wrong place. Uh, and so for our institution, we tend to use Restyin as sort of our bridge, and then if we want to do a longer term injection, we look at something like fat. So what about reinnervation? So interestingly, it's really not a new concept. Um, it was originally described around the same time when people were doing, uh, medialization, uh, laryngoplasty, uh, and it really probably didn't take off because it took too long for that to really get a good effect, um, and people were having a hard time getting sort of the same results as some people who were starting to advocate for it. Uh, there's several series in the adult literature. There's actually one randomized control trial in adults. It's a very small trial. Uh, there are some good learnings from that piece, which is it's better if you're younger and if you're closer to the injury. There are now several series in children with very promising results. Um, no real long term data yet. The largest series is actually Doctor Zur's, um, triologic thesis. So she was a former fellow from Cincinnati and she's got about 33 patients in that series with really excellent results for this procedure. So what's your candidacy? So, your ideal candidates are those who are within 1 to 2 years of injury, children, so less than 40 years of age, which fortunately for us, that's most of our patients. Although I will say I've done a 56-year-old who got excellent results from it. Um, knowing what the injury is, less ideal is those who you don't know where the injury is, um, greater than 2 years out from the injury, and then older patients, as I've mentioned. So advantages of a reinnervation, it is a general anesthetic. So for children, for, uh, you know, that aren't going to stay awake for a thyroplasty, this is a big advantage. Technically, it's a one and done. If it works, it's great and should last their entire lifetime. It's low risk. Uh, you can also always do other procedures later if it's not enough. It's their own tissue, which is a nice advantage. Disadvantages, we still don't have that long-term piece of data in terms of how these kids do once they get into adulthood. It sometimes can take about 6 to 9 months, sometimes even a year to get your final results. And interestingly for some of our families, this is the big no no, which is they really just don't want an incision on the neck, and sometimes it's just getting over that. So this is a video demonstrating what it looks like when you get the two nerves next to each other. You find a branch of the ansa going into one of the muscles. You need to make sure that it stimulates well. You find the recurrent. This is always the most interesting part is that you cut the recurrent, which we generally don't do unless you're doing a laryngectomy. Um, and then you hook the two nerves together. That's what it kind of looks like. Um, I usually put a little graft around it, uh, to allow that area to heal nicely as it heals, um, and to prevent little nerve sproutlets, and we do an injection at the same time. In terms of the voice piece, um, if you could. Again. So this illustrates a post reinnervation, and if you look carefully, you will note that the left side is still not moving. So it's probably a misnomer as an operation, which is, you don't get movement, what you get is tone and better closure, and oftentimes it's the outcomes in terms of the voice and swallowing piece that really move things forward. In terms of failure, so what do you do when it doesn't work? They're rare. You can always consider working up the patient for actually an arritinoid fixation and not a paralysis. Uh, you can think about long term injection until they get to be teenagers or young adults when they've already gone through puberty, and then you can look at a more formal laryngoplasty like you would do an adult. Just to illustrate an interesting sort of spin on this, this is one of the cases of one of my partners, Charlie Meyer. So 12-year-old autistic boy with a pilocytic astrocytoma of the brain stem, a unilateral vocal fold paralysis with aspiration after the resection. Uh, this is what his endoscopy looks like. You can see the pooled secretions, and one of the important pieces in this case is that just really poor sensation. Initially tried with just an injection with really no improvement. G tube tracheostomies were placed again for management of his aspiration and really no return of function. And then his early CT changes. Then when we look at what can we do next, um, there's an additional procedure you can do where you actually hook up the sens, the sensory component of the larynx. So you take the great auricular nerve and connect it to the superior laryngeal nerve to get sensation. Um, and this actually made a huge difference to this child. These are actually quite rare when they, you can find them, but it's something to think about when it's a sensory component is to think about doing a sensory reinnervation. Um, and it gives them some ability to recognize secretions and swallowing and move forward, and it's actually meant moving to a limited oral diet, which can be a real win for some children, um, and then looking at what their long term functions. So again, looking at using CT scanning as your way of measuring what happens with them over time. We, we haven't a big experience in the rural procedure in, in our country. The main hospitals of our city. No, not practice. The, the bilateral submandibular gland excision and bilateral parotid do ligation, uh, we use habitually the, the botulin's toxin A, uh, really, uh, we have a very good, uh, experience and most, um, 90% of the, the patient, uh, has, have, have a, a very good successful. Uh, with the ruling, so the, but the different series of the bilateral mandibular and um bilateral parotid delegation is a success. From 60 to 100%. Other possibilities some mandibular ligation for the treatment for the procedure. Um, is successfully to the 60 to 70% of the patients and recreation because we don't practice this, this kind of. Surgery. So and the nucleotic parotid scan diagnosis not use here and it's possible the relegation is a very good possibility for in this kind of surgery and the tympanic neurotomy. Uh, is the parasymphatic supplies to the parotid glander is sectioned within the middle ear, but the success is not uniform. I, I, we prefer use, continue with the toxin, the botulins toxin A for this kind of the patient. So it's, if you know, it's very good. Successful is probably the, the next step is this draw procedure or the Uh, the next slide with where you explain the, the, um. The separation of the, the larynx, no, the, the laryngotroal separation. We, we don't have, uh, we haven't the experience in the IIS with the four main hospitals, the pediatric hospital in our, in our country, and no, nothing use this kind of techniques. And the chief of the neurologist told me the same things. He said never need to practice the, the man the bilateral mandibular is the gland excision and, and forth uh dark ligation for the treatment of for yorrhea with enough with the. Uh, toxin in both limbs and tracheotomy with cat. For the Failures of a drool procedure, I'm going to briefly talk about laryngotracheal separation, and so a laryngotracheal separation, I guarantee of no aspiration, but you will not be able to voice either, and there's a temptation to think that you can put in a speaking valve, a Blomsinger valve or something like that. And really in children, this is not a very effective approach because the larynx is still on the way. And you've got to do a complete laryngectomy with a cricopharyngeal myotomy for that to have any chance of working. And so for most children with varynotracheal separations, you're dealing with children who usually don't have language because they've got significant neurological compromise. But they may be able to make sounds, and so that is a loss to a family, and so the, Technique we've developed in Cincinnati, next slide please, is to, Transsect the trachea, in Cincinnati we've done, Over the last 20 years, we do 1 or 2 of these procedures a year, so it's not a common procedure. So we've probably done 30 to 40 procedures over the last 20 years or so. And We initially found that there were potential complications with either fistula formation or with stomal stenosis, and we modified the technique. And at the moment, We do not have a problem. With fistula formation, we do not have a problem with stomal stenosis, and the things that we've done that are slightly different is that when we transect the trachea off the bottom of the larynx at the cricoid cartilage, the two things we do. Is that where the cricoid cartilage is, we peel the mucosa up, subperichondrial within the cricoid. And push it up like a sleeve within the larynx and do a purse string suture to close that mucosal layer that we've pushed up within the larynx, and then we split the cricoid cartilage laterally, 3 o'clock and 9 o'clock, and then we do quilting sutures to sew the anterior cricoid cartilage to the posterior cricoid cartilage. And put tissal in the subglottis, and then we divide the sternal heads of sternocleidomastoid. And crisscross them over the stump of the larynx. And so you end up with multiple layers of protection. You've got tissal and the subglottis, you've got the closed-off mucosa, you've got the sandwiched cricoid cartilage. You've then got the criss-crossed medial heads of sternocleidomastoid overlap between larynx and trachea. We pull the trachea up to the skin, split the sides laterally, and put skin flaps in, so you're doing a stomaplasty. And make sure we leave a tube in there in a child. In adults, you can leave an end stoma without a tube and it won't necessarily stenose. In children, it will steno, and once they stop growing, it stabilizes. So we leave a relatively big, wide, and relatively short tube in the trachea stoma. Since we've been doing that technique, we've not had any examples of a steal stenosis and we've not had any reconnections. And Here is a boy who's got lifelong aspirations, severe. He's already had a left pneumonectomy. He's had a draw procedure. He's had a deep intererotinoid notch noted. He's developing bronchiectasis of his right lung, and we don't want to take out the right lung because it's the only one he's got left. And. So We did an endoscopic. Laryngeal cleft repair for a deep notch. And he's still aspirating. So we've done a very successful repair of his deep notch, but the patient is a failure because he's still aspirating. It's more than just an anatomical problem he has. And this was after a laryngotracheal separation with a good outcome. He is now off oxygen, out of hospital, no longer aspirating, and his bronchiectasis is steadily improving. So one of the questions that we had submitted online was talking about, and this is for you, Phil, gastroesophageal reflux, PPIs, and what, um, do you feel about the use of PPIs with reflux in children who potentially aspirate. And there's been a lot of, uh, yeah, recent. Evolution and the thought about medical management with PPIs. So Phil. Right, so I think the issues that have been raised in the last few years with regard to PPIs are the risk of lower respiratory tract infection because of altering the microbiome in patients who have good quality acid suppression. There is certainly a risk for uh an increased risk for acquiring C. difficile infections in patients who are hospitalized a lot, which is certainly our population of patients. Um, for patients who have, uh, acid-induced complications of reflux such as esophagitis, uh, they're very effective. Um, the side effect profile beyond the infection risk, um, is really quite good. Um, so that, um, there's a balance between using PPIs appropriately at the right dose, at the appropriate time with regard to meals, um, to manage the complications of acid injury, um, versus using them as prophylaxis for whatever perceived complications of reflux might be. Um. We use PPIs at milligram per kilo per dose, um, once a day or twice a day. Um, there is emerging evidence to support the measurement of the patient's metabolism of PPIs, um, because there are some patients who require a substantially increased dose to achieve adequate acid suppression. Um, but as you've mentioned before, the, uh, use of PPIs doesn't alter the event of reflux, so that if the complications of the, uh, of the reflux are more a result of the bolus, uh, movement back up the esophagus, um, you're not going to achieve any resolution of that with, uh, PPI alone. And we're going to go through tracheoesophageal fistula, endoscopic and open, laryngeal clefts, endoscopic and open, esophageal strictures and pharyngeal stenosis, and we're gonna kick it off with another audience response question. And, uh, again, this is primarily for the surgeons, but please, anyone chip in. My preferred technique to manage tracheoesophageal fistulas is. Open thorascopic, divide the fistula and over sew it, tho thoracotomy, sorry, thorascopic repair, transcervical slide, endoscopic cauterization of the tract, customized approach to the individual patient. And really this is a question about does one size fit all. Of course, my personal favorite operation has had 0 votes, but I'm used to that. So, um. I think what we're gonna do is. Go and discuss some of the ways of managing tracheoesophageal fistulas and see whether we change anyone's mind. And so, tracheoesophageal fistulas can be your classical congenital associated with an esophageal atresia or a congenital H type. Or you can have an acquired fistula, the failed repair of a congenital fistula, and there is a recognized failure rate, a reconnection rate. A cuff injury from a tracheotomy tube, direct trauma. In America, we occasionally see bullet holes that will cause a fistula. Uh, a button battery is one of the more sadly, more common things we're starting to see. And the decisions, pediatric surgery versus ENT open versus endoscopic through the neck, through the chest, what an um, anesthetic technique would you prefer, particularly given that many of these children by definition have compromised lungs, particularly if this is a chronic problem. And the diagnosis can be surprisingly challenging. The history is usually very suggestive, and often we will be extremely suspicious that there is a TEF or a cleft and have to go looking for it. And the key here. Evaluation with a high index of suspicion. If you think there's a problem, you've got to go looking for it, be it a TEF or a cleft. Um, tracheoesophageal fistulas can be surprisingly challenging to find. And there's a lot of different tools and techniques and angled telescopes that can be of assistance. What you're seeing in this video is that we're using an angled telescope to visualize a very small tracheoesophageal fistula in the upper trachea or virtually just below the subglottis level. And just that little black dot is in fact a tracheoesophageal fistula. Being visualized with a 70-degree angle telescope. Here we've got another, quite useful example of a child who's had a TEF repaired and we're probing it. And we know that this child's still aspirating because they've had an esophagram. We've seen contrast go into the esophagus, uh, into the trachea from the esophagus, and we're probing the TEF repair, and that's a good TEF repair. And so you start looking. And again, these things can be surprisingly hard to find. The second congenital H-type TEF and there it is. And you can see it's not small, but it was not an easy thing to find. And so that's just replaying that same little segment. You can see distally, you've got a great closure. And you know the child's aspirating, so you go in with an index of suspicion. You want to look for the second TEF and As you know, this is using a bugby cautery as a probe. There's a groove which makes you suspicious, you want to probe that little divot, that little groove, and In it goes. Suddenly you see the fistula. Similarly, here's a girl who's had a tracheoesophageal fistula closed, and we're going to probe from the back to the front. So this girl's got an inadequate posterior cricoid. You can see she's trach dependent. You see an area that looks awfully suspicious at the apex of her past type 3 laryngeal cleft repair. And so how are you going to establish whether that is in fact a fistula or if it's just a divot. So this is using a probe showing she's got no posterior cricoid. Everything's very soft. This is using an alligator, and the alligator, as you open it, goes straight through the hole, so you know she's got a fistula, and this is the postoperative result after we placed a posterior graft and decanulated her. No fistula, adequate posterior glottis, quick look down the esophagus. And this is again a similar view with an endotracheal tube in the esophagus. And what we can do here is if you put a slightly, you know, a little air on a cup of an endotracheal tube. And you then look down the esophagus and the tip of the tube is proximal to the tracheoesophageal fistula, and you give a positive pressure breath, you should be able to see if there's any bubbling coming out of the hole. So now we're looking at the tracheoesophageal fistula or what we're suspicious is a tracheoesophageal fistula. We're giving a positive pressure breath, you'll obviously have to vent the G tube or stick a nasogastric tube in the stomach afterwards, and you can clearly see bubbles coming out through that hole, so that confirms it's a tracheoesophageal fistula. This is, again, a combination flexible bronchoscopy, esophagogastroduodenoscopy, where you can literally see one telescope looking at the other telescope through a subsegmental bronchus. Now, if you see that, there is no doubt that you've got a hole. And this is just a fun little video that one of our fellows made a year ago. I quite like the Star Wars effect myself. This is in a, my memory is it's a right upper lobe bronchus posterior segment. And at the same time, I think this is, uh, Phil, I believe that's you in the esophagus with an esophagus that is not entirely normal. And this is where you've got the EGD admiring the light of the flexible endoscope. And he. That's not normal. Oh hello. And I think that we Invited Dan Von Almen to kindly remove that segment of lung and fix the hole, which he did. Let's move on to endoscopic repair. And so the ideal candidate for an endoscopic repair has a long skinny tract. So it's usually a child who's had a recurrent tracheoesophageal fistula following a repair for a congenital TEF. And mostly these are proximal. Um, this is a more extreme one where we've got a significant tracheoesophageal, uh, fistula right on carina. In a child who's already had 3 repairs, nearly died during the 3rd repair. And Has had yet another recurrence and no one wants to go back into his chest. You can see all the suture material from one of the previous repairs. And again, the concept of an endoscopic repair is to demucosalise the tract. And let raw sit against raw. Mucosa is a non-stick surface, so you have to remove the mucosa, whether it's with trichloroacetic acid, whether it's with a bug be monopolar cautery, which is our preference, um, that's something you can steal off the urologists, generally speaking. That's removing the suture. You can see the esophageal mucosa there. We're now going to demucosalize the tract. With a Bugby electrocautery, which is a two French insulated wire with a live tip. And once we've demucosalised the tract, we're then gonna put a tiny bit of fiber and glue just to try and plug the hole temporarily. And then we'll uh. See the results a little bit later. So here's the bugby. And this is just demucosalizing the tract. In the interests of time, I'm going to move forward to the next slide before the tisseal goes in there. Same child six months later. And it is the same child. Can I ask what the biggest fistula you guys have been able to close endoscopically is? Uh, probably that one actually. That was a decent sized hole. And so we'll present another case. This is a girl who presents with an anorectal malformation and a type C tracheoesophageal fistula. Um, the esophagus and the fistula were repaired on day of life 3. And she was referred to us as she was clinically still aspirating. And our initial thought was, could she have a laryngeal cleft, because you should always go in with an index of suspicion in any child who's had a TEF repair and is still aspirating. Is there a laryngeal cleft? Frankly, I think that. When the child first has the TEF repaired, that's the time you should be proactively checking for a cleft. And so This is a video. Probing, looking for a cleft on the left hand side. And on the right-hand side, it's the bronchoscopy. So we're putting an alligator forcep through, checking for a cleft. On the right hand side, you can see that we are looking down at the. Suspected tracheoesophageal fistula, and this is the same video we played a little bit earlier. And so this is the first attempt of doing an endoscopic repair using a bugbe cautery. And so a bugby quartery is a monopolar guarded. Cautery with a live tip, usually we use about 10 watts power, and again a tracheoesophageal fistula conceptually, mucosa is a non-stick surface. If you can remove the mucosa, be it with a cautery, be it with trichloroacetic acid, your aim is to get raw against raw. If you can achieve that, you've got a good chance that the fistula will close, and so, This is the quartery as we're starting to, Explore the tracheoesophageal fistula. And we'll then actually. Start boviying, start cauterizing, you can just see that starting there. And this aim is to demucosalize the tract. And that's what's happening there. A demucosalis tract, it's worth actually coming up to normal mucosa in the trachea and just taking out a little bit of the normal mucosa at the entrance to the pouch, that seems to enhance the ability of the repair. Usually we put in a tiny bit of fiber and glue, that's what you see here being placed down a double lumen Julo catheter, and you need a very small amount. If you use too much it'll end up down a bronchus. And that failed. And you have to accept with this that you have a failure rate, and you have to be prepared to come back and do it again. So, endoscopic TEF repair, you should be prepared to do this on more than one occasion in some children. So here we are re-cauterizing it, and what we're going to do differently the second time to press raw against raw where you've demucosalised. Is we're going to inject an inert filler right beside the tract, so this is actually a temporary gel, radius voice gel that laryngologists use for vocal cord injections, and we're injecting a little bit in the back wall of the trachea, right at the TEF to squash it shut. And it doesn't need very much. It's surprisingly difficult doing this endoscopically because you're having to have a telescope beside your needle and they battle with each other a little bit. And this is the outcome. So this is this girl about 6 weeks later. And you can see the TEF sites completely eliminated. She's asymptomatic, her swallow study is normal, she's breathing well, and you can barely even see where the pouch was. Now, congenital H-type tracheoesophageal fistulas are sometimes not very symptomatic, so there's sometimes a late pickup. And again, you can see one here now. Generally speaking, with very few exceptions, these do not do well with endoscopic repairs. You typically have to think about doing an open repair. And there are different techniques for repairing H-type tracheoesophageal fistulas. And you can get to the upper 2/3 of the trachea through the neck. If it's the lower, lower third of the trachea, it's easier through the chest. The middle third of the trachea is really that sort of no man's land. It's whoever you've got who's better at doing it. And so whether that's pediatric surgery or ENT, I think it's very much the local, your local environment expertise. Whoever's the best person to do it should do it. And again, you can either do a transtracheal layered repair, a slight tracheoplasty if you're going through the neck, if you're going through the thorax, usually a thoroscopic or a thoracotomy approach where you identify the tract, um, ligated and transected. And so here's a boy who's had a previous TEF repair, and he's got a second proximal H type TEF. And so this is his anterior airway. That is his thyroid notch. And so he's got a very high fistula. And This is making an incision in the anterior trachea. And you do get fantastic access to the cleft. And this is just putting a probe through the cleft. We've got a bougie in the esophagus, so you can see, you're looking at the hole. And typically we'll split the hole slightly distal, slightly proximal, and then use a pair of super sharp scissors to connect those two incisions. So you're creating layers between the trachea and the esophagus. So this is just splitting the hole a little bit distally, just with a beaver blade, doing the same proximally. And then you can create layers between trachea and esophagus. You sew up the esophageal layer, knots in the lumen of the esophagus, put in a little interposition graft, that's the bougie you're seeing there, and then you can sew up the tracheal layer, and I usually again leave knots in the trachea. And that's the little interposition graft. In this case, we use some clavicular periosteum because it's sort of in the field. We quite often use sternal periosteum for this. And this is just sewing up the tracheal layer. With a simply enormous needle. I don't know quite what I was thinking at the time, but if nothing else, you can tell I was enthusiastic. And this is the post-op view, knots and lumen, they're dissolvable, and so most of the time we now use PDS. This is a relatively old video using viral repeat. What do you do about big holes? And so what we've increasingly been doing with very challenging tracheoesophageal fistulas is a slide tracheoplasty technique. And so this is an operation designed for complete tracheal rings, and we found that it's a very useful technique for a variety of other things, including challenging tracheoesophageal fistulas. So top and bottom, same patient, very large fistula from the cuff of an endotracheal tube before and after. Here's another girl. She's had 3 failed tracheoesophageal fistula repairs. She's got an associated subglottic stenosis. This is at the lower border of the cricoid cartilage, so it's high, pre-op, post-op. She's already had a transtracheal approach, a lateral approach, and a sternocleidoid uh mastoid interposition. And so here's a girl who was sent to us from Sweden. She's got. A type 3 cleft that was repaired twice. Ended up with a residual tracheoesophageal fistula repaired 5 times, as you can see the various techniques that have been used. She's had a T tube in place for 5 years. She chronically mildly aspirates. Um, this is with her T tube in place. We'll take the T tube out in a second. And, um, as you know, tracheoesophageal fistulas can be very hard to locate. But once the T tube's out, if you look very, very carefully, I think you'll be able to see the problem. Which is there. So that's called a big hole. And it's had a total of 8 attempted repairs. And so again, most of the standard techniques we use are not going to be very effective dealing with that. And so a slide tracheoplasty technique effectively means that you're transecting the trachea above and below the hole, and you're using the trachea attached to the hole to fix the esophagus and reconnecting the trachea over the top of it. The slide technique oversizes the trachea and gives you less tension through the suture line, we usually place a sternal periosteal graft at the same time. And so this is the girl as we're repairing her esophagus using some of the trachea on the esophagus, you can see the bougie there. This is where we're pulling together her trachea with a slight tracheoplasty technique. Multiple throws and again, it's very slowly tightening up the suture line with nerve hooks. And this just takes a little bit of patience. You really want to get this all together without a leak developing. And as we very slowly tighten that up. This is a quick look. Right at the end of the procedure, checking that we've got it all together. And this is intubating her through the repair at the end of the case. You can see the suture line there. This is her esophagoscopy. One month out And again, you should see some knots in the lumen. There And that's from the using a piece of trachea to repair the defect in the front of the esophagus, and this is her 12 months later. So she's Asymptomatic and this girl was a team approach because she also had cyclic vomiting, and after we've done that repair, if she had an episode of cyclic vomiting, she would have ripped it apart and she's very lucky. That our team included Phil Putnam, who's got expertise with cyclic vomiting. I think Phil, you remember this girl quite well. What did you do to stop her vomiting for a month? We didn't want her to even start vomiting, so the approach is more prophylaxis than waiting for her to start throwing up and then try to treat it. Um, so she went on amitriptyline as a preventative against cyclic vomiting. Um, and then she got potent anti-emetic drugs if she did start to complain of nausea, uh, that would indicate that a spell might be, might be starting. We're now going to present a case of a fairly challenging tracheoesophageal fistula. This is a 16 year old boy who was born with a pure esophageal treasure. He was a long gap. He did not have a tracheoesophageal fistula. He was originally repaired at 6 weeks old and it strictured. He had 30 dilations until at the age of 4, he actually ruptured his esophagus and ended up needing a thoracotomy, chest tubes, and an oesophageal stent. The stents were replaced repeatedly, and he coughed and aspirated until at the age of 14, a tracheoesophageal fistula was repaired with a muscle flap. And so when he was sent to us at the age of 16, he could not swallow saliva. He had been spitting into a rag for 4 years. He would wake up coughing every 3 hours at night and have to cough to clear his trachea to get back to sleep. He was GJ fed, and this is a developmentally normal boy. And so this is his initial microlaryngoscopy, bronchoscopy, esophagoscopy. And as we look at his trachea, He doesn't have any stenosis, but he does have saliva in his trachea and areas that look very suspicious for tracheoesophageal fistulas. This is now looking down his esophagus. This is going behind his larynx. And as soon as you get past cricoharyngeis there, You see, he's got effectively a completely strictured. Upper esophagus, he's got about 1 centimeter of esophagus and then you hit a complete stenosis. This is putting an endotracheal tube deliberately in his esophagus so that we can do a positive pressure breath and see if there's anything leaking in his airway. And again As we give a positive pressure breath, and as you push that tube, you can also get an idea on the tracheal side of roughly how far down the upper oesophageal pouch goes, you can see that there. And you can see that's where we are and just off to one side, you've got areas that look like they're probably fistulas, but we're not really seeing any bubbling. So they may be distal to the stenosis. And so this is his initial esophagogastroduodenoscopy from the top down. And again, you rapidly hit that wall with a pinhole at the end of it. So there is actually a very small hole there. And whether that goes anywhere useful, it's very hard to ascertain. And this is The esophagogastroduodenoscopy bottom up. This is going through his G G tube site and looking up, going into his esophagus through his lower esophageal sphincter, and again, you've got a very abnormal scarred esophagus with a series of holes, and which of these is in continuity with the rest of the esophagus and which of these are in continuity. With the trachea or represent blind pouches, it's hard to initially say. But a very abnormal short lower esophagus. And then we'll move on. So the question is, what are our options for this boy? We could do a cervical esophagostomy, a spit fistula, so that at least he's not having to wake up repeatedly at night and have to spend his life spitting in a rag. The other option would be to try and replace the diseased portion of esophagus, but doing a, you know, an end to end repair or doing a colonic interposition. would be challenging because effectively, what we found were several TEFs, about 3 of them. Connecting his esophagus to his trachea. We could try and bypass it by putting a piece of coal on. Substernally. And then your other option is to do absolutely nothing because he's sort of getting by, it's just not a great quality of life. And so what we decided to do was to bypass that segment of extremely diseased esophagus. Um, so we did a cervical incision and a laparotomy, placed the substernal colonic into position. So Dan von Elman worked from the bottom, I worked from the top, we sort of shook hands across his mediastinum. We didn't actually enter his chest. And he had a fairly tight nissan at the bottom, we left his esophagus in place because effectively, if we tried to remove it, we'd almost certainly take out the back of his trachea. And with it isolated, he's not going to develop a mucocele because it doesn't make much mucus and it will drain into the trachea to prevent a mucocele formation, and a little bit of mucus, a small amount each day, isn't going to cause him any problems in terms of significant volume. And so this is him after that procedure on the flexible bronchoscopy on the left, the rigid bronchoscopy on the right. And what you're seeing with this is, again, there are still multiple holes in the trachea. Going into the segment of native esophagus that's still there, you can see that on the flexible bronchoscopy beautifully there. So we know he's still got tracheoesophageal fistulas. But his esophagus is isolated at either end, and so he's not aspirating through these holes, and he's not gonna form a mucocele because he's got the holes. And he's now swallowing well through the substernal colonic interposition where we've connected his upper esophagus to the interposition connecting to the lower esophagus. So he's got an isolated piece of esophagus in there, which functionally isn't causing us a problem. This is his postoperative EGD. And again, this is going to connect into his colonic interposition. You're both passing through the anastomosis just there. And then you can see the typical redundancy of a clonic interposition. And the only problem he's had is that some years later, he had enough redundancy of his clonic interposition that we actually removed a bit of it and tightened it up. But that's really been his only issue. He's doing extremely well at this point in time. And so the bigger picture question, this is him 5 years out. Flex Bronc, MLB. And again, you can still see that he's got. Some holes in his trachea. But effectively He's got almost no airway symptoms at all. His bronchiectasis has improved, so clinically, he's doing very well despite the several tracheoesophageal fistulas still present in his airway. And this is his 5-year esophagogastroduodenoscopy. Again, the redundancy that we addressed. So is there any benefit to endoscopically try to cauterize those little fistulae or just because, I think it would actually be counterproductive if you blocked them all off, he'll then form a mucocele where his esophagus is, so they're actually useful, and he's now healthy, putting on weight, doing extremely well. And again, I think that's a, a useful sort of, uh, team approach to a very challenging problem. And so now what? The native esophagus remains in place. Do we remove it? Do we check it occasionally? How would you check it, or do you just ignore it? At the moment, we. Accept the risk that you potentially could have a problem with a neoplastic process, but his native esophagus is otherwise OK. It's draining into his trachea. There's no way of doing surveillance via imaging, and so we're just seeing him very occasionally, and we're not doing imaging at this point in time. Type 3 laryngeal clefts. How do you like to repair these? Transtracheal layered closure, lateral pharyngotomy, endoscopically or endoscopically unless there's a reason to do an open approach. And so this is an audience response. Wow. OK, well, we're sitting on 100% at the moment for D, and again, we've got a very nice mountain. Oh, OK. So we've had a 2nd vote, transtracheal layered, and lots more votes now coming in. There's a sort of a 20% lag with this, so you have to kind of bear with us a little bit. Not many people want to deal with it endoscopically, not many people want to do a lateral pharyngotomy. So at the moment we've got, Transtracheal open repair versus an endoscopic repair unless there's a reason to go open. Laryngeal cleft classification. This is the Benjamin Inglis classification. We've been nagging Andy for years, Andy Inglis, to actually modify that classification to include a type 4 long, because a type 4 cleft into the thorax is entirely different than a type 4 cleft that's approaching Carina or beyond Carina. And of course, Andy responded by retiring. So there's nothing we can do at this point in time. Um, Diagnostic dilemmas. The small clefts can be difficult to diagnose. So on the left, this is a flexible bronchoscopy being done by Paul Besh, who's about the closest thing to a savant with a flexible scope that I've met. And this kid so has a history that would go along with having a laryngeal cleft, and Paul is desperately trying to find it and utterly failing. And this is. One minute later, boy's got a type one cleft. Again, flexible bronchoscopy is not a good tool for evaluating a posterior laryngeal cleft. And clearly, A type one cleft on a rigid scope that you could not appreciate on a flexible scope despite looking very hard. And so endoscopic management. We're, the technique that we've developed in Cincinnati is a mass closure technique. It's the same concept with tracheoesophageal fistulas if you're repairing them endoscopically. You want raw against raw. Mucosa is a non-stick surface. Get rid of the mucosa. You want a wide strip of raw against a wide strip of raw. I'm going to pass on to Sandro at this point. So why then add in laser? So, again, just as Mike mentioned, it's getting rid of that uh non, that non-stick surface. So what's really nice about a laser is this is where you can take advantage of that part of removing all that mucosa broadly. So, this is using a KTP laser, you can use uh a CO2 laser, whichever laser you have in your armamentarium, it doesn't really matter which one it is. And you really want to do a wide swath in this area. Um, again, we find that a little, it gives you a little bit of hemostasis. Um, it's actually quite easy to do, um, and I think I find it's a lot easier to teach in terms of removing all the mucosa. Sometimes when you do these endoscopically with cold steel, you can get little tiny strips and leftover pieces. And this way, you just make sure that everything is sort of removed and out of the way. So then what about open management? So this is really reserved for your cases where you've got failed endoscopic repair. Um, so this may be for your, with type, some type 2s and type 3s. It's a Larena-Fisher approach. When we're thinking about our type 4s, it's a cervical approach, um, which Mike will talk about in a little bit. And then with our type 4 lungs, these are the patients that are especially challenging. So that means they give you anesthetic challenges, which may mean a double lumen tube, single lung ventilation, ECMO, or bypass just to do the surgery itself. They often have associated microgastria and multiple other congenital anomalies, and those pieces may actually drive your decision making about even attempting a repair if some of those other things are not compatible with life. And very important to counsel your families before even making the attempts that there is a very high mortality rate with these patients. Uh, if you look at the literature, it, it hovers around 50% mortality. Um, again, some of that's based on some of the older techniques. We talked about the concept of mucosa being a non-stick surface. We developed a technique for a basically a reliable, simple, fast method of endoscopic repair of a type 1, type 2, types, often type 3 laryngeal cleft, and a deep notch. Again, the concept is not an anatomical cleft, but a physiological cleft. We don't care if you've got a type 1 versus a deep notch. We care whether you're aspirating. And the suture technique we use in babies, I quite like to use a 60 PDS on a BV1 needle, but you bend the needle so it looks like a P2. In older kids, I use a 40 PDS on a P2. I think Sandra, you prefer to use an RB1, an RB1, or you can go 50 with an RB2, so it's the same sort of shape, it's the same needle as an RB1 except it's smaller, so it's a little easier to use. This is actually one of our fellows doing this, and we now do enough of these that we've really started to consider this a fellow level case. And this is real-time. And so this is a Fairly rapid removal of that V-shaped strip of mucosa. And Then putting in a couple of sutures. And so this is again close to real time. And this has become quite effective as the technique we use and we've done quite a lot of these. Do you have any idea how many of these we've done, Sandra? Yeah, um, in terms of numbers, I would say, well, at the very least, at least 20 if not more than that, I think we're way, way more than that actually, but um. The other key is release the area epiglottic folds when you're done because you're slightly cone up the larynx when you do this. And again, this is the post-operative view on a fees evaluation after a cleft repair. And You can remove mucosa. I like to use scissors, Sandro likes to use a laser. A tool is a tool, it's whatever works in your hands. And so open cleft repairs, we tend to be looking at the more severe clefts. So usually the fours, um, none of us has been brave enough to tackle a 4 endoscopically yet. Um, most 3s, we will try and do endoscopically unless there's a reason to go open. And so this is the transtracheal technique where you form layers between the trachea and the esophagus. You sew up the esophageal layer, knots and lumen, you sew up the tracheal layer, knots and lumen. You can use an interposition graft if you wish, wish to. And this is a coronal section of the same. And so here's an example. This is a boy who's got a cleft that is a type 4, but it stops at least 1 centimeter above Carina. And sewing it up, and this is the postoperative view again. Knots and lumen. Most of the type fours will end up with a tracheotomy. Um, one of our graduating fellows, Matthew Bergeron, who's now in Canada, has just done a type 4 cleft repair on a baby single staged, and I think that's the first time it's been done, really rather impressive. And so this is the same boy 5 years later. Now, we're gonna move through this fast. So, I was going to talk about a type 3 cleft with an inadequate cricoid, but we simply don't have enough time. So I apologize and I also apologize for everyone asking questions at the moment because we're just gonna run out of time for this. So, I do give you my apologies for that. We're going to blow through this to go on to something more interesting. So type 4 clefts. And the reason I'd like to talk about this is the type 4 clefts can be truly challenging, and we've dealt with at least a dozen. Um, we actually devised a new operation back in 2007, waited 4 years till we had a patient to do it on, and this is the technique we now use for the long clefts. We transect the trachea. At the lower border of the cricoid, we peel the trachea off the esophagus, so you right to beyond the cleft, and you can actually keep the patient intubated into one bronchus while you do this. And then you're looking down on the hole in the esophagus. You sew up the esophagus, you can do a second imbricating layer if you wish. You can lift up the larynx and continue the repair up to the tinoids through that approach. Place an interposition graft. Typically, we use sternal periosteum. You can use tibial periosteum. Then we sew up the back of the trachea with the with the endotracheal tube still in it, and then reconnect it to the larynx, intubating from above, reconnect the trachea to the cricoid, Let things heal, and then place a trach relatively late, 2 or 3 weeks later. And so I'm gonna show you an example of this. This is a girl who was referred while all of us were in Portugal, and the weather in Portugal was fabulous. It was a great meeting and we got a phone call about a 2.3 kg kid with quite a cleft. And this was the cleft. Now, this is a long cleft. So on the right, this is the right bronchus. No. This is esophagus. Over here, that's the left upper lobe. Still esophagus and if you look around. You can actually find the left lower lobe, so this is a cleft to the end of the left bronchus, that's left lower lobe. So as laryngeal clefts go, this is long. And We used the described technique to do the repair, and there are some nuances here. We waited 3 months to get the kid big enough to do this. So they were on TPN and we waited till they were over 5 kg. Almost all the kids we've repaired who were under 4 kg died. We had a team involved and Dan Ben Scooter, Katherine Hart, Phil Putnam, Sarah Zack, myself. We bronked this kid almost weekly keeping their lungs clean. They had a very big endotracheal tube sitting in the common hole, and we were not concerned about intubation because there's only one hole. You just blindly stick it in and it's in the right place. And at 3 months. And so with her, we actually did this on ECMO because it was easy. It was such a long cleft. We did an aortopexy as we came out. This is the trach. We had to put the trachea incredibly high because all of these children have a very short trachea. So the trach went through the cricoid. We're still sorting that out. And you've basically got right bronchus, left bronchus, left upper lobe, left lower lobe, and a girl who's not on positive pressure support, who can't eat yet but will get there, still has a trach, but will get there. With laryngotracheoesophageal clefts, the greatest risk is that the distal end of the repair typically may end up forming a tracheoesophageal fistula, that's the highest risk, and this is a nice example here. This is a boy who's had a type 3 cleft repair, and he's got a distal tracheoesophageal fistula. And so, and again, we're using a cuffed endotracheal tube in his esophagus. To show where the fistula is, and what this is, the 2nd slide or the 2nd video is after we've done a transtracheal 3 layer closure of extremely thin mucosa. This is what we would now do a slide tracheoplasty on, and he's still got a very small residual tracheoesophageal fistula. And so what we're going to try and do is fix this endoscopically, and this boy is 6, so this is difficult in a younger child. This is using a bugby cautery to demucosalize the tract as we've previously talked about. And we're then gonna place an endoscopic suture to ligate that tract and get raw against raw without things, uh, saliva moving through it or air moving through it. And this endoscopic suture. Let me just say that this is fairly edited because this is a difficult thing to do. And this is using a P2 needle on a 40 PDS and then tying the suture afterwards, a surprisingly difficult thing to do. And this is the postoperative view. 3 months later, you can see a little bit of the residual suture left, no tracheoesophageal fistula. Go on to the section on the dysfunctional esophagus, and this is talking about esophageal atresia, caustic ingestion, and button battery injury. And we'll do this in a case-based presentation, so the first case, JB is a 13 month old boy, slow feeder at birth. And did well until he was 6 months of age, but developed increasing problems with choking and vomiting when he started taking solids and. A microlaryngos, uh, sorry, an EGD actually looked pretty good with normal biopsies, a barium swallow showed some reflux disease, and an MRI scan showed an esophagus with a very thick wall extending the entire length of the esophagus. And he continued to get more symptomatic. He could only take liquids. He was starting to fail to thrive. And what is interesting and extremely relevant is his mother had been diagnosed with esophageal leomyomatosis and actually had a halomyotomy and a Nissan and was doing reasonably well. But you've got a mother and a son. With a very abnormal esophagus. And so he was referred to us. We did a triple scope as well as a CT scan of his chest, and so this is the CT scan of his chest, and that is a truly impressive esophagus. It's huge with a very abnormally thick wall. And this is his microlaryngoscopy and his bronchoscopy. And again, he's got Malaysia. But it seems to be secondary to the enormous esophagus behind the airway pushing forward. And while the malaysia is not its primary symptom, it's an inability to swallow anything bar fluids. He certainly has malaysia, the esophagogastroduodenoscopy. Again, he does not have a problem of stricture or size in terms of the lumen. He certainly has a problem in terms of function and his ability to clear his esophagus, and this is a very big esophagus. And so we discussed the treatment options. Should we do nothing? Should we just give him a G tube? Should we do what his mother had a halomyotomy with a Nissan, though his esophagus is markedly bigger than hers was, or should we do an esophagectomy with an interposition? Equally well, a gastric pull up. And we ended up doing an esophagectomy with a colonic interposition, and this is his esophagus, and it's simply enormous, lying on his chest. And these are the postoperative endoscopies, the MLB. And again, still Malaysia, but less Malaysia than he used to have. And then the flexible bronchoscopy. And again, a similar story. Still some Malaysia. But an improvement on what he initially had. And this is his postoperative EGD looking at his colonic interposition. As usual with a colonic interposition, you get a little bit of stasis and a bit of redundancy. But he's now eating and drinking well. Now, interestingly, with two family members affected, we actually worked him up for Alport syndrome, which is an X-link deletion of 2 genes, and in fact he does have Alport syndrome. And so now let's move into esophageal strictures. If saliva cannot get to the stomach, it has to go somewhere. And this is where drooling and aspiration can be a significant limitation to your quality of life. You cannot control esophageal, yeah, uh, secretion management without an adequate esophageal lumen. And so this is a video of a child with a tight esophageal stricture, 3 open repairs of an esophageal atresia. 6 balloon dilations, and what we're doing is injecting steroid into the stenosis. I then balloon dilated it with a non-compliant balloon. One steroid injection, one balloon dilation, having had 6 fails with CRE balloons, and he's never needed another dilation, and we're now 10 years out from that. Here's a boy who's had a caustic lie ingestion. He's got no esophagus. He hasn't got a stomach. He hasn't got a back wall of his trachea. What we're looking at here. Is is Coria and as you go past Carina. You can actually get down to his diaphragm. And what you're seeing that gray there, that's pericardium, that's the back of his heart. Being looked at through where his esophagus used to be. Actually, one of my colleagues in pulmonology went further than I was willing to go and got to the laparotomy class, which is quite impressive. And so interestingly, What we did was we placed a substernal colonic into position. And his trachea healed to his prevertebral fascia, he never actually needed his trachea repaired. It all just healed itself, and we were able to reconnect him with a colonic interposition so he can swallow. He still has a tracheotomy, but he's swallowing, he's talking, he's breathing well with a tracheotomy. Of course, the other potential problem area are pharyngeal strictures. And again, if you've got significant problems on the pharyngeal side, this may inhibit a normal swallow and predisposed to aspiration as well. And so this case presentation is of an eight year old boy who crashed a golf cart, had a head injury, was intubated for this, and at the time it was noted that he had subcutaneous emphysema. And was described as having a new Adam's apple. He was extubated and on extubation he had a poor voice with significant aspiration requiring a nasogastric tube and significant stridor and actually got discharged home like that without being evaluated. Uh, either with a bronchoscopy or an esophagoscopy. And he comes in 2 months later with this severe pharyngeal stenosis. Nasogastric tube, and you get through it and then you find his larynx. And You can see his vocal cords and ratinoid prolapse, as you go through his vocal cords, everything from there on in is essentially normal. He's a vulsed his supraglottic larynx off his larynx and developed a pharyngeal stenosis there. And so this is intubated through that stenosis and you can see this endotracheal tube is quite snug through that stenosis. We're gonna divide the scar tissue. And swing a flap to try and open up his pharyngeal stenosis. I do remember this was an extremely inconvenient day as I was in the middle of clinic when this all happened and had to cancel my clinic to repair this because this boy was on the verge of decompensating. And so we divide the scar tissue with a combination of scissors, sickle knife, and electrocautery using a needlepoint bovie. And you've got to be a little cognizant of where the carotid arteries are as you go laterally, but we're trying to unzip the severe pharyngeal scar and then swing a tissue flap in so that it doesn't reform. You're trying to get through all the scar tissue without going outside the pharynx and risking getting into the carotid artery. And this is just steadily opening up this whole area. And this is after we fully opened it up, you can now actually see his larynx. And this is after we swung a tissue flap in place. So you can sort of recognize most of what's going on there. And so certainly not a happy place, but much better than it used to be. Now, you know, this is going to re-scar, and this is him. With a rigid bronchoscopy and with a fees evaluation, and you can see his epiglottis is nowhere close to his larynx, it's a vast off, you go past it and there's his larynx. And interestingly, on the fees evaluation on the right hand side. It's very unusual, but he's actually got a safe swallow. And as we go to the next slide, this is him one year later. He's got a good voice. He's not snoring, he's got minimal exercise limitation, and he's eating and drinking an almost full diet with no aspiration. And we are now 10 years out, and he still has effectively no symptoms, no trach, eating and drinking everything by mouth, but still has a very unusual larynx. And so pharyngeal stenosis can be very difficult to fix. Recalcitrant scar is the norm. And while scar division and free flaps can be useful, sometimes you actually have to put in a free or a vascularized graft, a free flap. Sometimes you may need to dilate the area. The difficulty is, if everything's damaged, there's not much you can actually, Swing in in terms of a flap. And so for the severe pharyngeal stenosis, particularly in the child, children who've had a caustic congestion, I look upon it as a step ladder, and how many steps up the ladder can you get the child? What you're wanting is a child who's got a voice, who's breathing without a tracheotomy, who isn't aspirating, and who's swallowing without a G tube. And typically, you can't get it all. You can usually get them one or two steps up that ladder, if you're lucky, 3. It's very hard to get them with all of those things. And again, expectations matter. Counseling the family at the start is important. And so here's a boy who's at a cost of congestion. And he's got a grade 4 pharynx. It's a total block. He's had a recent tracheotomy. Glad he had a tracheotomy because he can't find his larynx anymore. And he's got a severe pharyngeal scar completely blocked after a caustic congestion and just injury. And so what we did was we punched a hole through, found his larynx. And what I started doing for these complex cases is putting in a composite stent. So I put in an endoscopic suprastomal stent that's extremely long. The top of it is typically sitting in the nasopharynx, and I wrap a sheet of silastic around it or beside it to make it supraglottically far bigger. And so you can't fit all of that through the vocal cords. So the suprasomal stent goes through the vocal cords, it's attached in the trachea, so locking it in place, and then you have a big sheet of silastic around it. And again, that holds all the raw areas open while they re-mucosalise. And so this has been a very effective way of dealing with severe stenosis. And this is his pharynx a year later. Yes, it's still extremely scarred. But you've actually got a connection through to his airway and actually through to his colonic interposition that we placed sometime later. He still has a tracheotomy. And so to conclude, pediatric aspiration really is a growing problem. We're seeing the survival of increasing numbers of children at risk, and aspiration maybe of food, drink, saliva, or even gastroesophageal reflux disease, and surgery may address anatomy or function. Another advertisement for our mobile airway card which is available on both Androids and iPhones, very useful if you do any sort of airway surgery, a photo of our hospital in Cincinnati, And this is the Cincinnati ENT airway team. And I would also like to just show another illustration of our aero digestive team as we discuss these complicated patients each week. And a final shout out to my friend and my colleague, Robin Cotton, who was instrumental in setting up the aerodigestive concept. He's literally just retired and without him, I don't think we would have achieved nearly as much in terms of the care of children with airway diseases and aspiration than we have today. And so an acknowledgement and gratitude to Robin Cotton, my friend and my colleague. Thank you very much.