Update Course Rewind: Updates in Achalasia 2023

GlobalCast MD, along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe. Hello, pediatric surgery family. I'm Cecilia Hihana, a research fellow from Cincinnati Children's Hospital Medical Center. Our 11th annual update course in pediatric surgery was held this past August. Today, we are talking about achalasia management. And for that, we have Dr. Mikol Petrossian. Dr. Wit Holcomb. And Dr. Timothy Kane. It's important is to understand what types of achalasias exist, so you can sort of set up for parents to understand how the disease progress will go. So, these are the types of achalasia that we encounter. They're all essentially the same. What what you want to understand is that pressurization at the LES pressure is very high on all three of them. They all act differently. So, in all cases of achalasia, the lower esophageal sphincter fails to relax at the right time. But depending on the rest of the esophageal movements, we have three types of achalasia. In type one, the esophagus barely contracts, so food moves down because of gravity alone. In type two, pressure builds up in the esophagus, causing it to become compressed. On the other hand, in type three, there are abnormal contractions on the bottom of the esophagus where it meets the stomach. Type three does not respond well to treatments. So that's why in our study, and then Tim will go over it, you will see that the recurrences happen much more in patients with type three achalasia. I'm going to go back to. I say also that, um, they all respond to, uh, myotomy of the lower esophageal sphincter, but the outcomes are a little different. So type two is the most common, but responds also the best to surgery. For classifying and diagnosing achalasia, we use the Eckardt score, which is the grading system most frequently used for the evaluation of symptoms, status, and efficacy of achalasia treatment. It's basically weight loss, dysphagia, chest pains, and the fourth one regurgitation. We use the Eckardt score to diagnose the achalasia with other things like manometry, and also use the Eckardt score to follow them clinically. Great. So, now that we touch the basics of achalasia, let's go through a case. We're going to a nine-year-old female presented with dysphagia, chest pains, weight loss, workup revealed type one achalasia. She was referred for consultation. So the question would be, what would you, what procedure would you recommend? So, looks like the lap Heller is going to be the number one, which, which is probably standard of care in pediatrics. Um, that's the tried and true, um, approach for achalasia. But there is a large, uh, an increasing experience in poem in the world. Um, you can't do robotic. Again, if you're comfortable doing the procedure, you should do that procedure, but as of today, I we believe that lap Heller is probably the goal standard with the procedures. Awesome. So, laparoscopic Heller myotomy is the first option for this patients along with the poem. But do we have to do a fundoplication at the same time? We currently don't do, uh, fundoplication. We do not offer any wrap. So in the in the adult literature, if a Heller is done, there's a fair amount of uh, good literature showing that you don't need to do a fundoplication. We have biopsied all the kids that we've done poems a year out. And with our current population, it's or the rate, rate, rate of reflux is about 5%. If you compare it to adults, it's around 50%. So, I don't know what what's the pediatric physiology is, but the reflux tends to be much, much less common in kids. So, can you remind us that that don't see it all the time, how detrimental to you at the time of Heller is the EGD dilatation Botox? Yeah, we have 50% of the kids had some intervention before, uh, whether it be, uh, not as commonly Botox anymore because people are learning it's it causes a lot of scar tissue, but many have had dilatations and it's pretty minimal in terms of fibrosis and things. So we don't really recognize it too much. The things that we do to see in kids who have had Hellers or poems before is there you got to get into a different plane because it's pretty scarred. So, currently fundplications are not recommended at the time of a laparoscopic Heller myotomy, as they can cause torsion in the esophagus and recurrence of the symptoms. Regarding other interventions, they have shifted away from Botox due to scar tissue, but dilatations are still an option. So let's just say you're you're doing a um a laparoscopic esophageal myotomy and you get a a little hole in the uh anterior esophagus. Would a uh an anterior fundoplication help with that with the healing or preventing complications related to the uh perforation? You can. I mean, other people have described doing that to seal the leak, but you can also put a couple stitches in it and be just fine. Great. So let's jump into another case. So we have a 15-year-old male who presented for evaluation. He has history of type two achalasia, status post Heller myotomy when he was 12 years of age. Continued to have dysphagia, underwent EGD with dilatation and Botox injection, and he continues to complain of dysphagia, weight loss, chest pain. He's currently getting feeds by NG tube. So, what is your diagnosis? What is the difference between recurrent achalasia and incomplete myotomy? I think uh an incomplete myotomy is basically, you know, not going far enough down. Recurrent achalasia, I would more categorize into growth, so someone who grows a lot. So if you have a symptoms in a child within a year, I think it's an incomplete myotomy. But recurrent achalasia could be so you do a young kid, a five-year-old, they're one and done, you never see him again, you think they're doing okay. That's probably if they end up getting it later, it may be recurrent achalasia based on growth. So, recurrent achalasia means the patient that had achalasia, resolved their symptoms after surgery, and due to growth, they present with symptoms again after a long period of time, meaning more than one year. An incomplete myotomy is a patient that never fully resolved symptoms or did it for a short period of time after surgery. So those are the three things that you have to commonly look if somebody comes in who had the operation before with giving the with the wrap and figure out what what's wrong with this child. Is there any of those three could be the the answer. So you have to investigate along with the manometry, EGD, biopsy. These are the steps we normally proceed. Is it esophageal manometry, EGD with an flip or GI referral? Manometry manometry will sometimes show achalasia. It's just never goes away. It's the same manometry. So we don't really send kids for manometry, we'll send them to confirm the achalasia if people come from different institution and the or equivocal readings are. End of flip is a uh machine that um it's a soft balloon, it measures the esophageal dispensability and also the diameter. End of flip is a tool that through endoscopy, you use a balloon to figure out if the myotomy was long enough. And it's just easy to use once you know the numbers and how to use it. So it measures the diameter of the esophagus, so before and after your myotomy. It also measures, there's accepted numbers for adults in distensibility index, which is the amount of pressure you need to distend the esophagus a certain amount, and there's accepted standards for normal in adults, and we extrapolate to kids and we we shoot for those numbers. So, time to summarize. First, we talk about the three types of achalasia and how each one has different behavior, but the same treatment. The best way to evaluate them is through the Eckardt score, and the diagnosis can be made with clinical exam plus manometry and endflip. The gold standard treatment is laparoscopic Heller myotomy, but poem is increasing in popularity and is better for recurrences. Also, it is not recommended to add a fundoplication. For recurrences, a thorough evaluation should be done to find the best treatment. Thank you for watching. 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.