QUAD #23: CHARGE Syndrome, Airway Considerations with Dr. Catherine Hart

Globalcast MD, along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe. Hi everyone. I'm M. Goti from Cincinnati Children's Hospital Medical Center. In October 2022, Cincinnati Children's hosted the quad conference, which was a combination of four conferences. The International Organization for Esophagealatresia, the Arodigestive Society conference, the Cincinnati Children's Airway Course, and the Cincinnati Children's pediatric dysphagia series. In this video, we're going to focus on upper airway obstruction and aspiration in Chart syndrome with Dr. Catherine Hart, an ENT surgeon at Cincinnati Children's. In a previous video, we explored what Chart syndrome is with Dr. Catherine Hart. To learn more, click the card on the screen to watch the video. What you see here is two different kids with Chart syndrome. When we talk about a chart larynx, there are a few things that we see. They tend to have big bulkier arytenoids. So you can see the arytenoids here at fairly well. They're oftentimes described as having laryngomalacia. And I would pause it that it's not truly laryngomalacia as we typically think of it, which is congenital laryngomalacia. In congenital laryngomalacia, excessive tissue movement leads to dynamic and periodic airway obstruction. However, in children with Chart syndrome, the issue is a structural difference with the arytenoids positioned more anteriorly, rather than a dynamic obstruction. It's just the whole structure tips forward and gives you one of the other characteristic features of the charge larynx, which is the foreshortened vocal folds. The vocal folds are not actually shorter. You just don't see as much because of the positioning of the arytenoids. They can have very difficult exposure and this is one where I typically make sure the family knows if they have a difficult to intubate child so that any provider who encounters them can be given that information. And as you can see here, this larynx is even more obstructed, so you really can't see the vocal folds at all. One of the other features that is very common and fairly universal in children with chart syndrome is some degree of feeding difficulty including aspiration. So when we think about dysphagia and feeding difficulty in charge patients, there are three big areas that tend to be impacted. Most kids with Charge syndrome, at least 90%, have some degree of cranial nerve abnormalities, which can greatly impact their swallow function. They also tend to have structural abnormalities, whether it be cleft lip and pallet or the upper airway obstruction. Especially as they get older, they have a lot of behavioral issues and many of those are focused on feeding. So it can be tough to feed these kids. These patients sometimes have a completely absent swallow. The secretions will sit on their larynx and not stimulate any sort of cough reflex. High rates of a reflux and then all of those things can contribute to aspiration. These fees are in kids with chart syndrome. You'd see the first one is fairly normal and you can get a nice view of the vocal folds. The middle one, little bit of impairment and the third one, you can see how the swallowed material just sits there in his ferx and at the level of the larynx, which is clearly a setup for aspiration. And we do see that aspiration is present in about 60% of kids with chart syndrome. So as we talk about the management of aspiration, you really want to consider all of the sources of aspiration. We all kind of default to the things that are going into our mouth. Oral intake is important, but you also have to keep in mind that kids are just as likely to aspirate saliva. Even if they're not being orally fed, they can aspirate saliva and they can also aspirate their own reflux. So the first thing you can do to decrease aspiration of feeds is to simply limit oral intake. And we would do this based on both either a video swallow study result or a fees result and the clinical symptoms. Some kids will respond well to thickening liquids or using a blend diet. And of course, there are the various combinations of NPO and feeding via tube. If you're going the medical route, you can consider Robinol, scopalamine or Botox. So glycopyrolate, when first started in a patient is typically 95% effective, but you have to increase the dose to maintain that efficacy and the side effects often lead to discontinuation as you get to those higher doses. According to Dr. Hart, sometimes you can make secretions really thick. So in kids who already have issues with airway clearance, if you thicken their secretions, you can significantly worsen the problem. And then as you get to higher doses, you can have urinary retention and flushing. Scopalamine can be very effective forria. It comes with a little bit of a delay in response just based on how it works. This one makes kids very drowsy and can make them unable to accommodate. In a lot of our kids with charge syndrome, they have pretty complex vestibular issues and when you add something like this into the mix, it can really be problematic. Botox also can be really effective. In this study of just under 100 kids, Botox was effective in about two-thirds of them. And unfortunately, did not have any impact on about the other third. Downside to Botox is that it's not a permanent treatment. The duration of effect is lasts on average about four months and so it has to be repeated. So it's oftentimes a good trial medication to see how kids respond and then you can move on to surgical treatment. And the goal here is to decrease saliva production in these kids. When we talk about the surgical options, some of them we don't typically do. Salivary duct relocation is more of a historical procedure and really not appropriate for kids who are aspirating because you're not really decreasing the production, you're just relocating where it's draining into the mouth. We learned from Dr. Hart that you can ligate the salivary ducts by doing a four duct ligation where you're ligating the product ducts and the ducts from the submandibular gland. Oftentimes, you can also consider excision of the submandibular glands. Typically we don't excise the prot, it's a pretty involved procedure for this goal. And then there's also the possibility of a tympanic nectomy when all these other things have failed. And that decreases salivary flow by decreasing the neurologic input for it. And often times what we'll see is some combination of these things. The four duct ligation only ends up with a 30% long-term satisfaction. Tympanic nectomy provides effective control, but it is a bigger procedure to perform. What we most commonly do is a drool procedure, bilateral protic duct ligation and bilateral submandibular gland excision, and it's a pretty common combination. Almost 90% successful. There are some complications. Primarily, we worry about injury to the marginal mandibular nerve, which can cause some impairment lip mobility and very rarely can make kids too dry, meaning no saliva. But I've only seen that a couple of times. Here's a tip. When all the options mentioned before are not sufficient or if you have a child with really bad upper airway obstruction and really bad aspiration, you have to consider the role of a tracheotomy. This is thought to be necessary in probably about a third of all kids with charge syndrome, usually for multifactorial reasons. Key to remember that the tracheotomy in and of itself is not preventing aspiration. Some people have the misconception that if you put a cuff trake tube in, that is protective from an aspiration standpoint, but that cuff has to be deflated and any of those secretions can still make it down into the airway. Trake makes it easier to maintain a good pulmonary toilet and to protect the lungs from material that is getting aspirated. We're more likely to have to resort to a tracheotomy in kids who have upper airway obstruction or chronic lung disease. And when all else fails, one of the only surgical options to truly prevent aspiration is to do a laringo tracheal separation. This is where we actually take the larynx and we separate it from the trachea, bring the trachea out through astoma in the anterior part of the neck and oversew the bottom of the larynx so that there is now complete discontinuity between the ferx and the airway. This will render children unable to talk. And according to Dr. Hart, they typically won't offer this unless these are the kids who are already non-verbal or neurologically impaired to talk. And we will typically only recommend this when we're seeing extreme impact on the lungs. In summary, Chart syndrome comes with common issues including airway obstruction, feeding difficulties and aspiration with many children having cranial nerve abnormalities. Management strategies vary from medical treatments like Botox and Scopalamine to surgical options such as tympanic nectomy and salivary duct ligation with severe cases sometimes requiring a tracheotomy or larigo tracheal separation to protect the airway. Thank you for watching this video. Don't forget to subscribe to the stay current MD YouTube channel. Follow our social media channels and download the stay current MD app for tons of content in pediatric surgery. Globalcast MD, along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe.