Surgucation ep.1 Pediatric Achalasia

Drs. Mikael Petrosyan and Timothy Kane from Children's National discuss what every parent needs to know about Achalasia.  They go in details about incidence, symptoms, and current treatments. 
for more information email us at info@surgucation.com
and visit us at  https://childrensnational.org/departments/general-and-thoracic-surgery

Transcript

Speaker 1 0:19

[inaudible]

Speaker 2 0:22

Hi, this is Michael Patricia , and I'm one of the pediatric surgeons at children's national hospital. I want to welcome you all to our new podcast series called surgery rotation . With a half of this series, you want to bring surgical education in a way that will make your child's care better. Once you know that you are never alone, we're hoping they will . We'll be able to educate you, bring about knowledge and answer questions and help take care of your child. I want to thank me, Han and Madera's family for strongly advocating for family education and sponsoring the series today. I want to welcome Dr. Timothy Kane to our first podcast. He is a chief of division at general thoracic surgery at children's national hospital. He is a world authority on the everything surgery, but specifically in forgot surgery. He is interesting forgotten here. He's my mentor. He's trained me. He's also a good friend and a partner, but , um, and we have interests both of us in foregut surgery today. We're going to discuss , uh , achalasia. Welcome Tim. Thanks for joining us. What is the achalasia and how

Incidence of Achalasia

Speaker 2 1:18

is it diagnosed? What is the incidence of ankle ?

Speaker 3 1:20

So achalasia is a neurodegenerative disorder of the esophagus, specifically the, the body or the Wally esophagus. And it affects the contractility in peristaltic or the movement of the esophagus, and also inhibits the lower esophageal sphincter from relaxing. So basically as you eat food soft , Gus should move the food down into the stomach. And as it , the entry, the stomach should relax

Symptoms of Achalasia

Speaker 3 1:45

and open up , um, in an achalasia. It doesn't. So it's a tight sphincter. So food gets stuck in the esophagus. So the initial symptoms may be a difficulty swallowing patients and kids specifically describe food, getting stuck , uh , they'll regurgitate food that they've eaten the day before. Uh, sometimes they'll lose weight because they can't really eat because they they're throwing up. And the other thing they can get is chest pain, which is , uh , abnormal contraction of the esophagus, trying to push food past that sphincter, which is not opening up. And a lot of

What tests to get for diagnosis

Speaker 3 2:17

times this

Speaker 2 2:17

Go on diagnosis because of the symptoms are so broad, right.

Speaker 3 2:20

And , uh , they get confused with reflux.

Speaker 2 2:23

So how do you differentiate

Speaker 3 2:25

What tests you get or what tests your parents ask for, or, or inquire whether it's pediatrician or gastroenterologists ? Sure. So, and I can tell you also that the incidence is much lower in kids. It's about one in a million. Um, although some studies are suggesting it's more common in an adult. It's a one in a hundred thousand people have the condition of a collision , not sure what causes it, but in what the presentation is, but in kids , um , 95% of kids under your age , your age will have reflux and they outgrow it. Um, but if you have reflux later on than that , um, you have to distinguish that from other soft gel conditions. And , um, uh , gastroenterologists would initially order in a Safa gram, which is a , uh , x-ray study where you swallow contrast, and they measure the , um, column of barium going into the esophagus into the stomach, and it should flow freely wind into the stomach. Um, and they look for reflux on that study , um, with achalasia, what you'll see as , uh , uh , narrowing at the sphincter where the contrast doesn't go through, it just sits there in the esophagus. So that's the initial study that's done, but that could also be found in a reflux stricture. If you have bad reflux, you can get a narrowing of the esophagus there, which is treated differently than a Malaysia . So the definitive study for achalasia would be in addition to it , doing an endoscope and looking at the lining of the esophagus where reflux strictures, what, what would be seen on endoscopy is, is a very inflamed esophagus , um, narrow opening, and just diseased from

Speaker 4 3:53

Most of the time, the gastroenterologist , the biopsy that to determine whether it's reflux related stricture versus just achalasia, cause you shouldn't have any changes in a soft G aligning with the organ lining , uh, in the achalasia versus a reflux stricture , correct. There's three types of achalasia is, and that will determine based

Types of Achalasia

Speaker 4 4:14

will be determined based on the [inaudible] . Yes. So

Speaker 3 4:17

Resolution manometry is the definitive test to diagnose achalasia in addition to what type there's three types. And so , uh, they all have a lower esophageal sphincter spasm or tightening doesn't relax. So Chicago type one , uh, has sphincter tightening and in consistent pair of stalls , this of the esophageal body type two , um, has a lower Satya sphincter spasm and then no Paracelsus. So the esophagus just sits there with little contractility and then type three has the , uh , Les spasm plus the , um, this coordinated really almost violent contractions of the esophagus, which contribute to a lot of the symptoms like chest pain and , uh, difficulty swallowing and things like that. But all three respond to , um, an interaction , uh , uh , surgical intervention on the, on the lower esophageal sphincter and , um, the two main surgical interventions. And the ones that we offer are the poem technique, the pur oral and the salvage, my myectomy , and a laparoscopic Hellerman

Speaker 4 5:18

Anatomy. I know as a surgeons, we have bias towards the surgical treatment, but what are other treatments that normally parents get offered before they come and see us? And then by the time parents come and see us, they're the sort of the end of the medical treatment or failed medical treatment. And they require surgical intervention. And we'll talk about it later, but there's a couple other medical treatments that they've offered that ended up undergoing before they're coming to see us. Correct. And what is your experience been with in kids with a Botox and balloon?

Treatments other then surgery

Speaker 3 5:47

So I would say the majority of kids , um , that we see a lot of them have had interventions unless we've been involved in their care. Um, our gastroenterologist or the mind set that most kids respond to surgical therapy and the, and some of the medicines used in all the patients are not effective. And, and essentially if you don't cut the muscle and you just use a balloon to dilate it, it's going to be temporary. If you have symptom relief , um, Botox injection works to relax the lower esophageal sphincter. That's also an endoscopic procedure done by GI doctors. And a lot of times GI doctors will say, let's do the least intervention here and see if it works. But , um , almost a hundred percent of the time it's only a temporary fix and for a child that has a long life ahead of them, a surgical myectomy actually gives long lasting , uh , results in better than 90% of kids. So by doing some other intervention first, whether it be a balloon or Botox injection, you can create scar tissue, which makes us subsequent surgery a little harder. Um, and we, we definitely have a fair share of kids. Who've had intervention before, and it's definitely difficult, more difficult to do than someone who's not in interventions, but yeah ,

Speaker 4 6:55

On top of that, there's risk of perforation with balloon. Correct. So that's also something that we always , uh , afraid of when we dilate ourselves that there is a possibility of perforating and we don't really, it doesn't really cut it, just tears everything apart and seems to merrily symptoms go away for , for a little while. And then it'd come back.

Speaker 3 7:12

Yeah. Generally for a balloon dilatation it's a month or less. Yeah, the Botox it's maybe a little longer, but almost always comes back. That's right.

Speaker 4 7:21

So there's two types of surgical treatments

Surgical Treatments

Speaker 4 7:23

that we offer at children's national. And , uh , Dr . Can collaborate a little bit more on a Heller myotomy um, this is done laparoscopically it's in a minimally invasive procedure , um, and is currently, I would say standard of care standard.

Speaker 3 7:38

Um, although now with increasing experience with the poem , uh, since 2010 , uh, you know, most, most surgeons will do either a laparoscopic or robotic Heller myotomy. Um, for kids, robotic surgery is not really , um, effective because the instruments are really large, so we can do laparoscopic surgery. And in the smallest kids we do for, for Heller, myotomy have been between three and eight months of age. So those are kids. We would do a lap Heller for, for kids larger than three years old or two years old. They're big enough that can, they can undergo a poem. So we've done kids in that age size and weight size in age range. Um, but the, the Heller myotomy basically comes from the outside and cuts the muscle. And then oftentimes you have to dissect around the esophagus so you can create a hiatal hernia or a , uh , reflux , uh , from that operation. So you have to do some type, usually it's some type of antireflux procedure and for , uh , uh, achalasia that antireflux procedures are partial wraps, which are by definition are less effective than a full wrap, but to do a full wrap around the esophagus, you're actually contributing to potentially difficult to swallow .

Speaker 4 8:50

So, so if the parents for those parents Rob means you would take part of the esophagus, the opera part of, excuse me, stomach and wrap around these Sophos , creating a sort of pseudo sphincter mechanism. So it prevents reflux acid and Valerie flux into the soft focus . But as Dr. Kane said, you break down the narrowing. You don't want to create another narrowing by wrapping the stomach. So the wrap is done in partial fashion, and it's really not that effective. And it's also controversial. Some surgeons don't even do wrap, right?

Speaker 3 9:22

Yeah. I can tell you that a lot of surgeons who deal with kids who are growing, who do a lot of lap Heller myotomy is , will actually not do a rap because of the kid grow a child grows. They can twist the wrap. They can, it can cause a point of fixation. And then oftentimes , uh , you have to go back in and take the wrap away, sometimes redo the , my otomy . So in our practice, we've gotten away from it because it's much easier to treat reflux than it is to treat the difficulty swallowing, which can be a big problem with, with anybody who's had a myomectomy initially, or is in , um, in terms of offering poem, it's B it's based on a discussion between us and the family. So sometimes we've had patients come in and say, well, they would prefer the Heller myotomy, but the majority actually are leaning towards poem because as you do the poem , um, you can actually tailor the, my otomy , um, based on the interoperative , um , balloon catheter, we reuse to measure the esophageal diameter and distance ability, and it's called an endo flip. And then also, if you do a , um, a poem, you can, it doesn't preclude going back and doing something else later, whether it be a repeat poem or a balloon dilatation, or even a Heller myotomy. But we have a series of kids who have had Heller's either here or elsewhere that have had dysphasia that we've gone back and done poems on and they've done quite well. So it's, it's a different , uh, aspect of the esophagus. So you basically have, you know , 360 degrees of the esophagus to work with. So , um, it's been

Speaker 4 10:50

For those who, for those parents, if your child had an operation or a medical procedure, whether it's being ballooned validation , um, Botox, injection , um , or Heller, myotomy the poem procedure, which is in our opinion, is one of the best procedures in achalasia is still feasible because it's, it's done through the esophagus, not through the abdomen. And it's basically like doctor cancer is a 360 degrees of tissue to work with. So if the Cod was performed on one side, obviously the Miami that's going to be done through point will be on the opposite side, that will be determined during the operation. So it is still feasible to do the operation, even though you had the previous achalasia operation that failed. If your child still has symptoms of the achalasia

Speaker 3 11:37

In our experience, we've not had to , um, revise any of our poem procedures to repeat my enemies . We've done a couple of poems and kids who've had poems elsewhere. Um, and some Heller ma um, had in poems and kids who hadn't myotomy so elsewhere. Um, but the most that we've had to do in some kids after poem was, is a single balloon dilatation. Um, and that that's sometimes breaks the muscle fibers. Cause after they heal, they can, they can create some scar tissue and then also with growth, correct.

Speaker 4 12:08

Uh , I think the important point for parents to take is that while it's great , uh , that we have with these procedures, it's important to understand the achalasia itself doesn't really go away, correct? Correct. It , it doesn't go away. We just treat the symptoms, right? So your child gets better. They go have normal life, which is very good. It's phenomenal. But the, but to say with [inaudible] , it goes completely away. It's really not true,

Speaker 3 12:36

Right? The esophagus will always have abnormal contractility , um, there's little information or data to suggest that the esophagus heals are curious, but what we're treating as a symptomatology , um, we we're preventing weight loss. We're allowing a normal life because kids can gain weight and they can eat normally, but the esophagus will always have abnormal contractility. What we don't see in kids, which sometimes happen, which happens often in adults, as they get such a dilated esophagus that they adults go on to end-stage achalasia where their soft Gus needs replacement. We don't have any, there's no data or there's no info , you know, literature about that happened to kids. So we think it might be different in kids . You actually respond better to surgery because if you do it early, you , you have a chance to kind of allow the esophagus to grow and heal and hopefully not need

POEM

Speaker 3 13:26

interventions in the future. Like some adults need correct.

Speaker 4 13:31

You get how the procedure done and what it entails. Sure.

Speaker 3 13:35

Um, procedure involves putting a , um , endoscope in the esophagus under general anesthesia, which is a camera it's just like the endoscope they likely had when they had their , um, endoscopy by their GI doctors. And then we , um, take measurements of the esophagus to calculate where the sphincter is. Um, and we're where we're going to make the incision in the soft geo wall about, you know , 10 to 20 centimeters above the lower esophageal sphincter. And then we insinuate the camera between the mucosa, the esophagus, the lining inner lining of the esophagus and the muscle. And then we dissect all the way down to the stomach. And then once we had that created that artificial tunnel and we can see beautifully where the muscle is. And then we just cut the muscle from the stomach side up to the esophagus side. And usually that's anywhere from a five to seven centimeters. And then at the time of surgery, we use a balloon catheter called an endo flip balloon, which measures the soft deal , um, lower esophageal sphincter diameter, as well as the just sensibility. And then we can , uh, pretty carefully calculate how much we need to cut that muscle and how long to make , uh, uh , relieve the difficulty swallowing. And initially what we see classically on the balloon catheter is that the diameter of the esophagus is about instant millimeters, like five to six, seven millimeters. It's tiny. And I show the parents the pictures before and after it's like an hourglass. And then after it looks more like a straight tube or just a mild waste , um, in , uh , across the sphincter. And it's over 10 centimeters typically, which will , um , tell us that we've cut the muscle enough. And then after cutting the muscle and we are happy with the balloon catheter measurements, we clip the lining of the esophagus closed and then a child wakes up and the next , uh , does not, has not given any, anything to eat

Post-øp Course

Speaker 3 15:28

or drink until the next day we do an, a soft gram basically just to calibrate the esophagus in case down the road, there are symptoms. We can always repeat the soft gram . And then , um, typically that we , we get that baseline study. We haven't had any leaks or any, any problems with that study. So it's basically a baseline. And then we advanced the diet on clear liquids and then basically a no chunk diet for two weeks, but most kids will go home then a day after surgery or two days after, depending on how far they live away from the hospital or the area. And if they're tolerating diets and not needing IV fluids and things. And in terms of pain, most kids don't complain of any pain. Some will have a sore throat from the breathing tube and the endoscope. Um, but that's pretty much the , um, the most common complaint, but not significant.

Speaker 2 16:15

We do have a lot of patients who came through here and you want to tell a little bit to our listeners.

Speaker 3 16:21

One of our , um, one of our parents created a basically, because no one knew about

Parents of Achalasia

Speaker 3 16:26

achalasia in , in her area, in her state. She created a Facebook page called parents of achalasia kids. And she's been a champion for , uh , getting the word out about , um, a achalasia and there's there's , um, patients and families on this Facebook page, which include adults and adult patients basically as a , uh, a chat and support group, because in a lot of areas, no one's ever heard of this. And particularly in her , uh, instance , when her son got her upper GI, the radiologist said, he'd never seen this before. And we see it all the time here, but you know , we're in a big center in a big city , uh, but a lot of places out away don't, don't see it. And if you don't know what you don't know, if you haven't seen it, then you may not know it. So she's been really a champion of creating this , um, Facebook page

Speaker 2 17:13

Testimonials. There you can, if you're interested, you can go to parents of achalasia on Facebook, find their group and sort of register and inquire. Uh , if you have any other questions regarding the procedure, or just in general, you want to talk to us about the procedure or anything else, feel free to contact us through children's national.org is the website. You can also directly email us at info at [inaudible] dot com and the link will be right below the video as well as the podcast. Um, I want to thank you for being here, Tim, hopefully this, like I said earlier, this won't be our last conversation. And we're hoping that with this podcast, we'll be able to teach many of you out there about the disease. We do have those procedures here. We have great experience, come and visit us, and we'll be happy to answer any other questions if you have in the future. Thank you and have a great day.

Speaker 1 18:55

[inaudible] .