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 Gigena, 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 accolade management. And for that, we have Dr. Mikkel Pratrosyan, Dr. Whit Halkom, and Dr. Timothy Kane.

It's important to understand what types of accolades exist so you can sort of set up for parents to understand how the disease progress will go.

So these are the types of accolades that we encounter. They're all essentially the same. What you want to understand is that pressurization and the LES pressure is very high on all three of them. They all act differently. So in all cases of accoladesia, 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 accoladesia. In type 1, the esophagus barely contracts, so foot moves down because of gravity alone.

In type 2, pressure builds up in the esophagus, causing it to become compressed. On the other hand, in type 3, there are abnormal contractions at the bottom of the esophagus where it meets the stomach. Type 3 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 3 accoladesia.

I would say also that they all respond to the myotomy, the lower esophageal sphincter, but the outcomes are a little different. So type 2 is the most common, but responds also the best of surgery. For classifying and diagnosing accoladesia, we use the Eckhart score, which is the gradient system most frequently used for the evaluation of symptoms, stages, and efficacy of accoladesia treatment. It's basically weight loss, dysphagia, chest pains, and the fourth-hand arm regurgitation.

We use the Eckhart score to diagnose the accoladesia with other things like manometry and also use the Eckhart score to follow them clinically. Great! So now that we've touched the basis of accoladesia, let's go through a case. We have a 9-year-old female presented with dysphagia, chest pains, weight loss, workup reveal, type 1 accoladesia.

She was referred for consultation. So the question would be, what procedure would you recommend? So it looks like the lap heller is going to be the number one, which is probably standard of care in pediatrics. That's the tried and true approach for accoladesia. But there is a large, increasing experience in poem in the world. You can do robotic. Again, if you're comfortable doing the procedure, you should do that procedure.

But as of today, we believe that lap heller is probably the gold standard for the procedures. Awesome! So laparoscopic heller myotomy is the first option for this patient, along with the poem. But do we have to do a fund application at the same time? We currently don't do fund application. We do not offer any rap.

So in the adult literature, if a heller is done, there's a fair amount of good literature showing that you don't need to do a fund application. We have biopsied all the kids that we've done poems a year out. And with our current population, the rate of reflux is about five percent. If you compare it to adults, it's around fifty percent. So I don't know what the pediatric physiology is, but the reflux tends to be much, much less common in kids.

So can you remind us that don't see it all the time? How detrimental to you at the time of heller is the EGD dilation botox? Yeah, but we have fifty percent of the kids had some intervention before, whether it be not as commonly botox anymore because people are learning it causes a lot of scar tissue. But many have had dilations 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 see in kids who have had hellers or poems before is you got to get into a different plane because it's pretty scarred. So currently, Fanta applications 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 dilations are still an option.

So let's just say you're you're doing a laparoscopic esophagal myotomy and you get a little hole in the anterior esophagus. Would an anterior Fanta application help with that with the healing or preventing complications related to the perforation? You can. I mean, other people have described doing that to seal the leak, but you can also put a couple of 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 2 achalasia status pole heller myotomy when he was 12 years of age. Continued to have dysphasia under when EGT with dilatation and botox injection. He continues to complain of dysphasia, weight loss, chest pain. He's currently getting feeds by NG2. So what is your diagnosis? What is the difference between recurrent achalasia and incomplete myotomy?

I think an incomplete myotomy is basically not going far enough down. Recurrent achalasia, I would more categorize into growth. So someone who grows a lot. So if you have 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 OK. That's probably if they end up getting it later, maybe recurrent achalasia based on growth.

So recurrent achalasia means a patient that had achalasia result 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 a 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 a rap and figure out what's wrong with this child. Is there any of those three could be the answer. So you have to investigate along with the manometry, EGD, biopsy. These are the steps we normally proceed. Is it a sophogram, manometry, EGD with endoflip or GI referral?

Monometry will sometimes show achalasia. It 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 institutions and or equivocal readings are. Endoflip is a machine that it's a soft balloon. It measures the esophageal sensibility and also the diameter. Endoflip 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 the kids and 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 Eckhart score. And the diagnosis can be made with clinical exam, plasmonometry and endoflip. 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 fund application. For recurrences, a thorough evaluation should be done to find the best treatment. Thank you for watching.