Persistent Abdominal Pain - Median Arcuate Ligament Syndrome: Update Course 2014

Uh, we're gonna talk a little bit, uh, mostly about minimal invasive, uh, treatment of median archi ligament syndrome, which is a great topic for the group because many don't believe that it exists and maybe they're right. Uh, but, but the context is an adolescent, a female that's been, had over 100 blood tests, been scoped, been scanned, they have abdominal pain, and they're sent to you and They literally have a terrible quality of life. They've left school. They can't participate in sports or any other activities, and they're in front of you asking for some help. So that's really the context. This is kind of somewhat rare and and highly uh uh uncommon and unusual even for abdominal pain. But, uh, let's talk about it. So it's median ocular ligament syndrome. Others call it C-like arter compression syndrome. They're, uh, synonymous. And the pathogenesis is unknown, which is a good way to say no one knows what is going on. Whether it's mesenteric ischemia, if that's real or not, or whether it's neurogenic stimulation and, and, uh, by releasing those nerves or cutting those nerves, are you helping? But, uh, the thought is that there's compression of the celiac artery with the median ocurate ligament. So usually, the presentation is that of abdominal pain. And traditionally, in, in textbooks and articles, it's adult literature, they say that it's uh postprandial intestinal angina or postprandial abdominal pain. But that's really not the case with uh kids. With adolescents, we're finding more and more that it really is post-exercise intestinal uh uh angina or abdominal pain. That's actually something that pops up more frequently than just purely the, the post-op, the postprandial abdominal pain. Weight loss, not so much. That's, uh, again, an adult issue, but, uh, they have a little bit of weight loss, nausea for sure, and some vomiting as well. And the problem is it's really a diagnosis of exclusion. So again, the context is that of a teenager or an adolescent that's come to you and has had truly an extensive workup, whether uh at your institution or elsewhere. They often have had upper endoscopies where the, uh, with biopsy, they've had lower endoscopy to look for inflammatory bowel disease. They've had ACT and then a CT enterography, and then perhaps a nuclear medicine study, and gastric emptake study or their gallbladder. They've had abdominal ultrasounds, uh, CTs or MRI's. And then they're asking you, can you just take out her appendix? Is that gonna work? Can you take out her gallbladder? We don't know what to do. Or sometimes they've even come and they've had their appendix or their, uh, gallbladder taken out. And really, the question is, are you gonna entertain a diagnosis of exclusion or entertain whether you're even gonna think about median archid ligament syndrome doesn't exist? Are you gonna go after this as a diagnosis? And if you do, what are you gonna do about it? So really to make the diagnosis, what you'd like to do is be able to get some kind of angiography and magnetic resonance angiography is great. Um, very minimal uh radiation, it is technique based, so it should be at an institution that does these often. Um, you do, you can do CT angiography. Those are much easier for the surgeon to interpret. Uh, they're also very quick, so you can do the inspiration and exploration, um, uh, at the same time. And you do want to make sure, this is a critical point that when you're trying to think about this, making this diagnosis, at least anatomically, you do want to have a dynamic study where you get images on the CT scan during inspiration, and then you get a CT scan during expiration with CT. It's easy. Cause you can scan the entire body in 20 to 25 seconds. So that's something that the adolescents can cooperate with. And then you wanna make sure that, that you get a duplex ultrasound. The velocities, the range on the, there's a, there's a difference on the lab that you're at. But above 300 centimeters per second for sure is suggestive. Um, different labs have different criteria. And you wanna be able to compare that postoperatively so that, uh, you don't need to get another CT or MRI, uh, afterwards. So this is a CT scan. This is uh what it looks like anatomically. Um, you do just simply have a critical stenosis, um, of the celiac artery. You get a compression. Often, you will see poststenotic uh dilation. So that's what you're looking for anatomically and when, when you're asking for the study. And this is just a, a pre-op angiography again, uh, uh, with 3D reconstruction. This is what the ultrasound looks like. Again, you want a lab that does this quite often. Uh, these are just little details, but you do wanna make sure that you get that study. And when you're trying to look up, well, is this really gonna work or not, is it, you know, there's uh quite a bit of uh adult literature, but in the pediatric uh literature, There's very few studies. Uh, in fact, there's basically two that have, uh, looked at this with very small, uh, amounts of data. But the highlights really are that they have gotten an extensive workup. They have a clinical scenario where they do have abdominal pain, uh, that's postprandial or post-exercise. They are highly selected patients. Um, and the studies basically show that there is, they're safe, uh, when done in experienced hands or experienced centers. And they do have some efficacy or at least improvement of quality of life. So if we can just show the video just so that we know the procedure that we're talking about it, um, you can do this open as well, but I haven't had to do that. Uh, so this is the. Uh, gastropatic ligament that, uh, is being opened up. The approach is much like a Nissan. This is the cruise. If you stay right anterior on top of, uh, the cruise, there are no vessels that you should hit other than the aorta. So that's what you're looking at. You're gonna uh go right on top of the aorta to find it. That's at least my approach, uh, is to find the aorta and then go, uh, towards the feet, um, to find the, uh, artery itself. So you're dividing, uh, these, uh, muscle fibers. And you essentially go millimeter by millimeter uh to make sure that you do not make a mistake and injure the celiac artery. As you're doing this, the arteries compressed. So you really don't see it unless you, until you start releasing some of the ligament. And again, this is just going millimeter by uh uh millimeter. So until you release some of that ligament and uh cut some of the nerves, um, you really won't see the, the artery. But now you can just see a hint of it, uh, just behind the, the cautery. And again, it, this is a little bit tedious, uh, but you do wanna be careful cause you're 1 millimeter away from opening up an artery that cannot be fixed. Easily. Um, and there's really no issues of stenosis in adolescents that I know of, uh, in the 40 to 45 year olds plus, there are, uh, stenosis that you need to fix with patches, but not in the adolescence. So again, you, just take your time as long as you uh know the anatomy and you completely release it, uh, almost 360 degrees, and you end up with uh an artery that's, uh, free, and that's the technical goal. Is it something that we can technically do? Yeah, I'm sure most of the people in the room, if they're, uh, good with, uh, laparoscopy, then, then they can do this. But that's really not the question is, can you do it? The question is, should you do it? And my approach is really, if you have a team that's able to support you and the patient, then maybe that's something that you, it's something that you can uh consider. So I often plug them into the pain service or even uh psychology, even before we do the procedure so that afterwards, uh, they have some sort of uh support. And usually this is done in conjunction with your gastroenterology because functional abdominal pain is something that's very difficult to treat. Uh, if you, uh, if you're treating these patients, because often they'll go a different way. They'll have inflammatory bowel disease or other things. Then there's a question of mentorship. OK, fine. You have a patient, you're gonna do this. You're gonna just try this by yourself. Are you gonna have a vascular surgeon available? We can talk about that a little bit, but that might be an issue of even for people that are, uh, uh, competent. Are you just gonna try this on your first or second time by yourself? And that's a question, so we'll conclude that wrap this up quickly. Um, overall, minimal invasive surgery is safe for, uh, median archal ligament syndrome. I think that's been borne, uh, borne out, at least the safety of it. Can you do it? The patient selection is crucial. Uh, they have a fast recovery. Um, they leave the next day. So you have a procedure Monday. By Tuesday afternoon, they're asking you to leave. They do really quite well, assuming that no intraoperative complications occur. Quality of life is improved in the short term, uh, uh, um, but we don't have any long-term data just yet. And basically, is this something that when you have persistent abdominal pain, are you even gonna entertain the diagnosis or not? Um, That's really the ultimate question after that, there's other things like mentorship. So, Abdullah, do you get, do you do intraoperative ultrasound? I don't. I mean, I, I played with it once and I didn't find it that useful. You're gonna release the artery. So if you know the anatomy, and you have your, uh, so again, I don't find the left gastric artery and then try to go back. I, I don't do it that way. So, I mean, this is the same technique that I've used, but I, you know, talk about mentorship. I, I did my first one with one of our vascular surgeons who ironically doesn't, does, do minimally invasive surgery. But he called me up because he had a 14 year old that he was pretty sure had this and was consulting on her and she had a very impressive, you know, the 3D reconstructions are very impressive. And he, he came into the operating room with me and sort of watched over my shoulder as I did it. It's a, it's a little nerve-wracking, but it's not a difficult operation as long as you don't get into the artery. Yeah, you don't get into the artery. It's not, it's, uh, yeah, you gotta be careful. You gotta be careful. I mean I've spoken with, with colleagues that have gotten into an artery of some kind, and it's a disaster. Yeah, it's a disaster. Yeah, yeah, so, yeah, yeah, so we've, we've, we've seen videos in our, uh, complication session at IPEG of, uh, someone getting into the artery. I mean, it's, it's, he's a good surgeon. I, uh, I had the opportunity to be working at the Comer Children's Hospital when Don Liu, who I think did the first series of them there. He was a superb, minimally invasive surgeon, superb, uh, but the whole trouble with this is that people have for years try to deal with chronic abdominal pain in children. In the old days when, uh, you know, Jay and I were in the, uh, in the trenches there. You'd get somebody with chronic abdominal pain back and forth, and you go in and you take out their appendix, OK? You close the belly, and a huge number of them got better. You knew damn well they didn't have acute appendicitis, and the appendix was normal. So my problem with this, I watched, uh, Don do it. It's a pretty thing to do it. It's not hard to do if you're good with the sticks. But my question is, there's no, no, uh, data to show that you're doing anything. It's like the massive increase in taking out gallbladders for poor emptying in the last 10 or 15 years in kids, you know, some children's hospitals have done 50 or 60 cholecystectomies a year now. We did one a year in, in my era. So I, I, it's interesting, but you have no data whatsoever, whatsoever that this has anything to do with their abdominal pain. If you're going to do this, you've got to have some kind of control study to look at in which you just open the belly or just put a bunch of scopes in the belly getting that to the IRV. Yeah, well, put scopes in. We have always said that there is just this wonderful collateral circulation in children, so it's hard to imagine that this stenosis is going to be an actual etiology of the pain. And the question, so that begs the question, if for some other reason you got this study, you know, post trauma or something like that, and you saw this anatomy in a child with no symptoms, what would you do? So is that, is, in other words, is the symptom, is the so-called stenosis or the, the anatomic change you see, is that in and of itself pathology? So I, I agree with, with the, with both comments. So to answer the, your, the latter question, I have been referred patients that are at least on the phone, look at the CT scan. It looks like the arteries compressed, compressed. I'm like, well, how's the kid doing? And they said, he's doing fine. I was like, well, then they definitely don't need to see me. And please don't refer him to someone that may accidentally operate on him either, because that's not the right thing to do either. You are treating the patient. And as far as the data is concerned, I mean, uh, you, you, I, I personally think that in these controversial issues like this, it's best done and it, and it should be studied. I mean, at, at, uh, my institution that at the ones that, that we published, every single one, including the first one was done under, under an IRB protocol. They were prospectively studied. Uh, we put them in a database. Someone followed them before and after. We had questionnaires, validated questionnaires for the, for the children that 87 questions for the parents, 50 questions asking about quality of life. There was a significant difference in almost all the parameters for pre and post, but the follow-up was low. What percentage of the patients improved? 75 to 80%. I just give them the follow-up study and I'll do the surgery and see how they get better. I mean that's a possibility as well, but they've had, um, I mean that's not something that you can do in real life, but they have often been at this for 1.5 to 2 years. I mean I how many did you do? I mean, I've, I've done, uh, 21 so far and, uh, in Chicago, uh, like you said, and, and, and done. Uh, Don's group, uh, they published 46 patients. So how many of the surgeons in this room, there are 10 or 12 people sitting there. What's the cumulative experience with this operation? I've done, you said you've done. One, I've done one. I've never done one. I've done. I've never done one, but I've watched a few. So 32 got better, 1 got better, and then got worse again, and she didn't have any compression. Did you give them the follow-up study, the papers? Yeah, I did, I did not, um, but you know, the, the first one that I ever did, the mother actually came to me with the diagnosis. I hadn't even really heard of it. She, this, her child had had abdominal pain for, and, and she had worked it up, and she was looking for a good laparoscopic surgeon to. Do the operation and I reviewed it all and everything else had been ruled out. Did she find a good laparoscopic surgery? No, anyway, you know, but she, but it, it's an, but it's interesting because then you do have centers like in Chicago where they seem to have huge numbers, you know, what I consider to be relatively numbers. Well, first of all, we don't look for it. I mean, people aren't looking for it in their abdominal pain patients, you know, getting the vascular study would be. Uh, you know, maybe the study that needs to be done is send all your abdominal pain patients, but then send normal controls and see if there's a difference in the populations with, with the anatomy, and you can do it. You can do it without a CT. You can do the vascular lab and look at the celiac artery velocity. I think the number, I think you had it up there was 300. You know, if it's, uh, velocity is over 300, there's significant compression. I think they in Chicago, it's interesting now they're not doing anywhere as many at the Coma Children's Hospital as before. Unfortunately, Dan, Dawn is, you know, passed away, but, uh, so, you know, it, it, you, you see what you're looking for sometimes in medicine rather than looking for what you need to find, and, uh, I, I think I, I don't want to be critical, but this is sort of a An open area, I, I think that the chronic abdominal pain child has bugged all of us, and it just depends on what area you were working in as to what the remedy, the remedy of the, of the year was. Used to be an appendectomy, then it was a cholecystectomy. Now it's this, the trouble is, an appendectomy is no big deal laparoscopically. A cholecystectomy is no big deal laparoscopically, but if you do 100 of these and get one hole in a celiac artery. How are you gonna justify that and you lose the kid on the table because I think that's a real possibility. So of the, should we poll the audience to see how many, uh, people have done it and poll our folks we didn't ask, uh, Dan and And Philip is 00 Samir. I haven't had to do it yet. And then Philip had to go to the OR and Sean. Well, I think I read one thing you always remember is that a patient who has abdominal pain and doesn't have anything wrong with them, the only thing worse than seeing that patient is when you put a scar. On that patient. Because then every pain gets focused on adhesions and all those kinds of things. And the other thing I would say is these patients, when they come to the office and you give them, start a workup of a diagnosis, they hang their hats on that diagnosis. So, my question to you would be, you know, some of these kids sound like they lose weight. I mean, stuff that really might be more pathologic or the celiac, um, artery velocity, do you have a couple things you really say, well, this is what has to be positive before I'd consider this as a diagnosis? So they need to have the clinical presentation before we even undergo that route, otherwise I see abdominal pain. I'm like, no, there's, you know, it doesn't fit. And they must have uh the anatomical features of the syndrome. So, uh, in our, I mean, in the lab we're at before, it was 275. It was 250, they just increased it. But 275, 30 whatever it is that your lab decides. And they have, you have to see the stenosis. If they don't, then what do you, you know, what do you, you don't know what you're operating on initially. But if they don't have any critical, if they don't have stenosis, then you're not operating on them anyway. And many have come with the diagnosis. That's what they're told. And you look at the studies and you say there's no stenosis, so, you don't do anything. And then the counseling is, you know, crucial, you know, I've, I've had very satisfied patients because you tell them, these are the risks, this is what's involved. If you make a mistake, you're 1 millimeter off, it's a disaster, you can get X, Y, Z and die. And so a few patients like, well, that doesn't seem like it's worth it. Great, not for you, perfect, go away. And others say, you know ones are worth studying to see what happens to them, correct, correct. Those those patients are, uh, and, and so the opposite is true where if you are studying them and you know, we, we just have the short term data on them, but we're, um, if you follow them out to see does it really help or not so that you can have some data to say does it make a difference or not, and if it doesn't, great. Um, and if it does, then that might be something that's useful. Abdul, I want for just a minute here because I also want to just talk about abdominal pain in general, and I know that that Ernie, uh, talked about this before. I'm just curious, and I'm trying to figure out the best way to do this rapid fire. What are people's algorithms for perambilical abdominal pain? Well, we get an X-ray, see if they're constipated. Do we get EGDs? Do we get colonoscopies? OK, those are all negative, or do people not get them? When do you get the CAT scan? When do you take out the appendix? Would you take, when do you get the CCK HIDA? What do people have? I know there's a great article on chronic recurrent abdominal pain that goes through this algorithm, but I'm curious if anybody has a, you know, most of the time those, all those studies have been done. They show up to me after GI. has done everything they can possibly think of and then it's kind of like, here you fix it. OK, so now they've had, I have, I have basically you do enough tests until you find one that's abnormal and then you call that the diagnosis. When would you operate? When would you, so you've had a negative CT, negative EGD, negative colonoscop. s c op y it's like a diagnostic laparoscopy or right, when would you put a scope in? Well, no, you're doing an appendectomy. No, would you put the scope in and not take out the appendix? And at this point I'm sending him to Abdala. I mean, clearly he can work miracles. Is there someone? Is that when you guys would go to the OR? I wouldn't even take out the appendix today. I would say in my experience, uh, taking out the appendix that, you know, was irrelevant. I, I would, but you just said before how many people got better. They get better from just opening the belly, but I think part of this is patient management and helping that patient move on. If you've had a patient who's been having abdominal pain for a year, they've been to GI, they've had an upper endoscopy, a lower endoscopy, they've had a HIDA scan, ultrasound, CT scan, and they found nothing. Then they're sent to me and I say, well, I can tell you, I can do a diagnostic laparoscopy. I think the morbidity is very low. We can talk about whether or not we take your appendix out. I said every once in a while we find sort of a chronic scarred appendix that we can't really tell on the outside. Um, I usually take it out because if you don't, then they will, that will always come back up in the thing. And it's a, it's a, and if they, if you go in and you don't find any adhesions and you don't find it, anything else, and you take out the appendix and they still have pain. Then you can help them move on to a pain management strategy as opposed to looking for a cure of their pain. We did a study, we, we did a combined study, uh, wrote it up with one of the GI guides at the, at the University of Colorado, and 70% of the kids that we did that in got better after the surgery. But there was a recurrence rate, and I can't remember what it was, but a certain percentage ended up having pain again in 6 months. But I do think that at some point, because the morbidity is so low, I look at it as another diagnostic test, and it's the only way to help the family take that next step to say there is not. You know, there is not an anatomic or, you know, reason for this, and we need to figure out how to help you deal with this and kind of get on with your life as best we can. 111 test that has cost you absolutely nothing is the history. One of our senior GI people said, ask them if they're going to school. He said all of his Crohn's patients go to school. They never miss school. If you ask your chronic abdominal pains, well, you've been going to school, you'll find the ones that are, are, are probably are functional have missed months of school with all these tests and everything, they get all this secondary gain. And so if they are going to school and seem to really have a good history, I think I'm much more aggressive about working that patient up. But that's what he, his perception was and it's worked for me for 20 years. Dr. Clapworthy had a rule, and this goes back 50 years. Kid comes in with chronic abdominal pain, put him on something like MiralaLax for a month and see him again at that time, right? You almost never saw the kids back. Yeah, I, I was gonna say that of all the things I've done for this, getting something to soften their stools was the most effective. Every one of these kids seem to have a little bit of, uh, subtle constipation. On that note, speaking of Crohn's disease, yeah, on that note, I think, uh, I think we'll move on.