The Colorectal Quiz Episode 9: Motility Disorders Part 2

Hey there listeners, this is Amanda Jensen and Rogerardo from Cincinnati Children's. Have you downloaded the new version of the state current pediatric surgery app? The reason we bring it up is because if you're listening to this podcast in the app, you can look at X-rays, colostograms, articles, all in real time. Well, Doctor Fisher and Doctor Levitt are talking about them. So download the app today. It's in the Google Play Store and in the Apple App Store. But until then, enjoy the episode. So last week we had a really interesting case. Amanda, do you want to help me recap it? We talked about motility disorders, specifically discussing a 10 year old male. This poor kid had chronic constipation, soiling, poor appetite, and then we talked about if they come into the office, we got to do a workup, and what's that? Including a history, physical, what diagnostic imaging? And we spent a lot of time talking about the utility of a water-soluble contrast enema, and that enema showed the torturous and redundant colon that was dilated and full of stool. And then we got anorectal manometry which showed an absent rare. This blew everyone's mind that the patient had this imaging and an absent rare, that's where we left off. So without further ado, this is the colorectal quiz. Crickets. I hear crickets waiting for. It's amazing. It's amazing that in February, that's Mark Levitt from Children's National. In the Midwest in Washington DC you can hear crickets because it's, there's snow outside. What are the crickets here? I really think this patient should have the, uh, you have to assess the colonic transit and you really have to assess the colonic function. That's Anil Darbari. He is a pediatric gastroenterologist at Children's National Hospital. Uh, it is not purely a sphincter issue. I feel that this patient is showing massively dilated colon as a result of the colonic dysfunction, although the dilatation is predominantly in the rectal sigmoid. Uh, we really have to assess the colonic function and provide some sort of mechanical modes of remedy for this. Yeah. I think also, uh, I think also, um, I, I completely agree, and Neil, uh, doing a SIS marker study. There you have Kalib Graham, Peds gastroenterology at Cincinnati Children's Hospital. More than just finding if there's a rare or not, but actually, what is the resting pressure? So what if the resting pressure was high? That might be a patient that might be amenable to something like anal Botox, for example. And then You can have the patient bear down. What is that test? Conscious rectal sensitivity threshold, essentially. OK. How do you do that? Gradually blowing up the balloon and getting a sense when the patient actually has a sensation to go to the bathroom. Because what's normal physiology? Is you're supposed to push from your belly, increasing your intraabdominal pressure, and also relaxing your bottom, creating a positive pressure. But sometimes these motility disorder patients have a negative pressure instead. What we call um dysynergia. And so those types of patients might actually benefit from like pelvic floor physical therapy or biofeedback. And those words of wisdom are really the foundation of understanding anorectal manometry. You know, you did mention a sits mark study. Do you do the sits marker study on therapy? That's Jason Fisher from Cincinnati Children's Hospital. Or do you take patients off therapy to do the SIS marker study? When do you get an X-ray? At day 5. It's how I was, how I was trained. Uh, so the patient will take the SIS marker, and then at day 5, they get, they get an X-ray. I'd like to use the SIS marker as a, as a screening test, and that helps the GI doc determine when and if I'm gonna do colonic manometry, if I see a lot of sit markers scattered throughout the colon or predominantly on the right. Side of the colon, that it sort of piques my interest a little bit more pote to potentially do a colon monometry. But if all of the markers are just sitting at the bottom of the colon, then it really is, fits more with an outlet issue. And then what if all the markers have magically disappeared? Then the, then the patient did stool, even if they told me they hadn't stooled in, you know, uh, in a month, right? They don't dissolve. They don't dissolve. So really, you use the sits marker study to tell you whether or not the patient has a motility disorder, has a distal outlet obstruction, or has somewhat normal stool evacuation. We have a lot of colleagues throughout the world. You know, this is obviously an international forum, and I think a sits mark study should be available pretty much anywhere. I can tell you that colonic manometry is absolutely not available everywhere. I find stitch marks could be utilized in various ways. I was trained with an adult gastroenterologist who also trained me that you could utilize this as a colonic transit study in the form of assessing colonic function. And you do that by getting your X-rays at day 0124, and then, Uh, you can actually see the transit of the markers in the colon. However, the cyst marker study is not a replacement for clonic manometry. But like Doctor Levitt said, let's say you're somewhere out in the world or the community that doesn't have the capabilities to do anorectal manometry. Most of them have nuclear medicine capacity, you know, they do renal scans and things like that, and there is a really nice way to do a nuclear centigraphy. Surgeon's perspective, what I need to know is one of 3 colonic motility scenarios. Number 1, is it diffusely slow? But pretty good. So like if the car is stool and the freeway is your colon, if I were to drive from Washington DC to Baltimore, going 50 miles an hour. I get there eventually. It's annoying, but I get there eventually. Option two, motility is normal, but segmentally, you have a problem. So in Doctor Levitt's car stool analogy, there's a traffic accident for 2 miles. During those 2 miles, I'm only going 10 miles an hour, but the rest of the time I'm going 70. Lastly, scenario number 3. Is the entire colon ridiculously slow? And modal. That's like me going to Baltimore and going 20 miles an hour the whole time. As much as these four guys love centigraphy and sits marker studies, keep in mind that anorectal manometry is still the gold standard. It's just a really sophisticated, expensive way to diagnose, and it's not available to everyone else in the world. So, let's get back on track to anorectal manometry. So why don't you guys tell us a little bit about it. Yeah, I think, uh, you, you, again, both of you made very valid points and, and often I say. Complimented us again. Well, that, OK, that's never happened ever. All right, come on guys, let's focus. The colonic manometry, I think, is additionally giving us information on the peristaltic activity, which is basically the motion of the colon. So when a doctor asks, how are your bowel movements, they're really asking, how is your colonic. Movement. People don't say that. We just say we have bowel movements. Throughout the day. You should have two really strong contractions throughout your colon. Those are called high amplitude propagating contractions. So if we can measure the pressure from the cecum all the way down to the rectum. Then we are able to see these contractions starting on the right side of the colon in the cecum area, and then going proximately in a progressive way. And if the patient has those two contractions throughout like an 18 or 24 hour study period, then you do not have a colon. dysmotility because presence of HAPC will essentially rule out colonic slowness or problems, and that's not uncommon. We have a lot of patients that get colonic manometry, but the results come back normal. Well, I guess we should define normal. No one normal or typical gets a colonic motility test. So those patients are more along the lines of like really slow transit. Does the colon move pretty uniformly throughout? Are there HAPCs throughout? Because I know then that antigrade flush is going to work well. Reliably in that colon. I, I, I, I agree mostly with that statement. There are some patients who they have, they truly have an outlet issue, and their colon is actually normal. So on the manometry, not only can you characterize the degree of contractions that a person is having, but you can also characterize if the, if the contractions are well coordinated. So is there a more of a neuropathic problem, or is there a low amplitude issue and there's Actually a myopathic problem. And then you could see where the contractions are happening cause they should go from the right side of the colon all the way down to the rectum. Your rectum does not have those same contractions. And then consider a Hirschprung's patients whose status post at PSAP, they had their rectum, their anus removed, and it was redone by the surgeon. They no longer have that rectal sigmoid break, and you actually might see contractions going all the way from, uh, the, the, the right side of the colon all the way down to the sphincters. But the amount of information that these GI docs get from the anorectal manometry is incredible. Dr. Darbari went on to say that you could even test how they respond to stimulants, even if there is a presence of HAPCs and you think the colonic motility is normal. If you do not see a response to stimulants, then by definition that patient does not have a normal colonic manometry or. Normal colonic motility. Jason, what do you think about maybe us trading our gastroenterologist because I, I like, I like to believe a lot. Maybe we could flip them. How about a rotation? How about we put them on a rotation? It's like our pitching staff. I want to get to the, the crux of this case and explain what we did. Um, so basically, we concluded that this was not Hirschprung's, and as Jason said, it was a sampling error. I was so convinced that I actually didn't re-biopsy because of the calretinin positivity. And then they went on to the anorectal manometry, which showed that the colon was diffusely slow, and they were pretty convinced that the problem was the sphincter. Cause we had the absent rare situation. And they thought the colon still needed a little bit of a kick. So we offered this patient a Malone for anti-grade flushes in combination with Botox and biofeedback physiotherapy. Over time, I, we think that this colon could probably rehab, and then eventually they may just need just laxatives alone, but I think a perfectly good endpoint. Is to have a mechanically emptying colon. I used to be someone that if you can't get them on laxatives, it's a failure, they need surgery. I don't think that's true. I think enemas from below, if they're willing to tolerate it, or enemas from above, and I put that in quotes, through a malone or a sycostomy, and if they fail that therapy, they may then need a resection. But keep in mind, the patients who fail this conservative management are the ones who have slow transit throughout or they have segmental disease. The vast majority. of those with the segmental disease respond to antigrade only. And I remember the vivid moment in Columbus when I was there, where Richard Wood and I and Carlo Di Lorenzo and Desiaco all said, listen, as of January 1st, every patient, no matter what the motility, we're gonna give them antigrade and see how they do, and we're gonna track them. And the vast majority of patients responded to antigrade only. And never needed a resection. Because prior to that, remember, Doctor Levin, Doctor Fisher, remember taking out colons that in retrospect, probably just had motility disorder and they didn't need a surgery. Now, here's a closing thought from Dr. Darbari. What we learn from each other is beyond what textbooks can teach us. I think a collaborative model like this should be promoted in every center where we provide care for colorectal cases. Oh, you're still here. You must be waiting for the weekly joke. I'd hate to disappoint, so here you go. Why didn't the toilet paper cross the road? It got stuck in a crack. Uh, I like that one. Oh, terrible, uh, awful. So there you have it. The second part of our motility disorder podcast in our colorectal quiz series, we had some great discussions with our pediatric gastroenterology colleagues. They taught us a lot. Hopefully, you learned a lot. If you did, if you Loved it. Please let us know. You can leave a comment if you're in the app. Download theta Current pediatric Surgery app. It's in the Apple App Store. It's in the Google Play Store. You can leave comments. You can start a discussion with pediatric colorectal surgeons across the world. Download it today. But until the next episode, I'm Amanda Jensen and Rogerardo from Cincinnati Children's. And remember, knowledge should be free.