Hey there listeners. Welcome to another episode of the Colorectal Quiz. I'm Shimon Jacobs, colorectal surgery fellow at Children's National in Washington DC. Today we have a very special episode on perianal Crohn's disease, and we're going to be referring to a few images relevant to the case. If you haven't yet downloaded the Stay Current app, go to the Apple App Store or the Google Play Store. It's the easiest way to follow along with this podcast and also access hundreds of other resources in pediatric surgery. Now let me introduce our first host, Doctor Jason Friser, director of the Colorectal Center at Cincinnati Children's. How are you feeling today? I'm excited today because for 2 reasons, actually 31, we're doing a little bit of a different topic today. We're excited about that. 2, I'm getting to reconnect with some old friends. And 3, my former colorectal fellow is on the line too. So, this is really exciting, a little bit of a reunion of a lot of friends of mine. Uh, without further ado, Mark, you ready? Ready. That's our second host, Doctor Mark Levitt, Chief of the Division of Colorectal and Pelvic Reconstruction at Children's National Hospital. Here today to share his expertise. And now, let's welcome our distinguished guests. First, we have Doctor Lisa McMahon, who is the surgical director of the IBD clinic at Phoenix Children's Hospital, and she has brought with her, her pediatric surgery fellow. Uh, Christine Velasco, who I always had the pleasure of training a few years ago at Cincinnati Children's, and now she's showing off her talents at Phoenix Children's Hospital. OK, let's get into it. Here to kick us off with their interesting case is Doctor Velasco. So today, we're going to be talking about an 8-year-old male who presented to us with 3 days of perianal pain in the emergency room. He had previously been, being worked up for IBD at another hospital due to a history of diarrhea for almost a year, some abdominal pain. And while he was supposed to have his uh colonoscopy and EGD done, he ended up having it canceled and presented to our hospital due to fevers. His perineal exam showed a lump that was deep to the skin and focally tender. The ER had obtained an ultrasound of the perineum, which showed a complex collection, 2.5 by 3.5 by 2.5 centimeters with hyremic soft tissue. Let's Talk a little bit about this. What I'm hearing is an 8-year-old that's already being followed for Crohn's, that's never had any surgery, and now has something wrong in their perineum. Is that, is that correct? Correct, not officially diagnosed with IBD. And, and just out of curiosity, just from a radiology point of view, you, he had an MRE. The outside MRE showed 30 centimeters of inflammation of his terminal ileum. What's the colonoscopy for? For the. Pathological diagnosis. Uh-huh. So what do you, what do you actually do? What do the gastroenterologists do? They're going to take biopsies throughout the colon, terminal ileum, and then an EGD as well. OK. So, I just wanna make sure, I mean, there are a lot of people on this call or listening to this podcasts. The colonoscopy might be normal for the entire colon, right? Even the biopsies might be normal. The duodenum might be normal. And the money might be in a biopsy of the terminal ileum, but what if they can't get into the ileocecal valve? How do you make a diagnosis? You do a capsule study. You can also send fecal uh calprotectin. Uh-huh, OK, good. So, so we need to make the diagnosis. We're, we're seeking a diagnosis here, and then this poor kid comes in with a very significant perianal problem. And by the way, the ultrasound, the ultrasound they did was, was just of the perineum or they did a. Uh, we don't. Routinely, I, uh, really get transrectal ultrasounds. Do you get those? We have to distinguish what the adults, the adults do it all the time. We have actually a, a, a GI doctor who has a scope that has transrectal ultrasound, and we have actually the wand transrectal ultrasound, um, just starting to learn how to use them though and how it works. But anyway, going back to this case, Mark alludes to some great problems, and if you can't intubate the terminal ileum with your Uh, colonoscope, then you really don't have a tissue diagnosis. Then you have to go to the assumption you have Crohn's disease, treat, and then re-scope after a few months of treatment, where it likely inflammation has gone down and you could, uh, intubate the terminal ileum and get tissue for true pathologic diagnosis. But that's a very Stressful situation for our GI colleagues because then they're using medications without the true tissue diagnosis which we always want to have. Jason, would you do a diagnostic, or Lisa, would you do a diagnostic laparoscopy in that case? Jason and I trained, both trained at Mount Sinai, where Doctor Crone was. Um, and so there was a lot of IBD. Um, and I remember, and maybe I'm dating myself a little bit, but the, the Barry Sakey recommendation in that case was to take a look at the terminal ileum laparoscopically. Is that something, not something we would do? Not on purpose. I wouldn't do that on purpose. Um, I think, I think there'd have to be something really huge pushing me that way. Or if it was an accident, going for something. I think back in 1998, Mark, when Remicade first came out in the market, people were a little more fearful of, of, uh, prescribing that as they are now. Are people no longer afraid of Remicade or biologics? I think people are less afraid of them. Although in this case, it actually factors in because these will, you'll hear, the parents are very, very afraid of Remicade, and I think it has to do with how they were counseled initially. This is really important to know for all pediatric surgeons, gastroenterologists, trainees, and everyone taking care of children with IBD. What are some of the real risks associated with biologic agents? So, uh, infectious complications, like, you know, rare risk of getting, you know, tuberculosis. Uh, we usually will send a quantiferon for that or PPD, uh, risk of lymphoma. I think that is one of the ones that tends to scare, in my experience, scare uh families and parents the most. More often, the highest risk factors of males, teenagers, and in combination with methotrexate. So, the, but, Those are just higher risk factors, it, it is a black box label to all patients, and, and that's why there's some hesitancy to get a tissue diagnosis to make sure you're treating the right thing when you're putting a patient at risk with something like this. Lisa, am I right in thinking this is a pretty atypical case that has both TI and perineal disease, basically at the same time. I, I think it's actually pretty common to have perianal disease and Crohn's, and he just has a delay in diagnosis. And then he's actually had perianal disease for quite some time. He's had multiple little skin tags and fissures. No other abscesses that we know of or that he could tell us. But I don't, I don't think this is a really atypical. Thing for him. OK. So let's, let's hear more about the case. So he was initially admitted and started on IV Cipro and Flagyl. Then the next morning, we took him to the operating room for an exam under anesthesia, possible incision and drainage, and possible seatone placement. Here's a very important learning point. How do you know when to take the patient to the OR? Do you have enough information at this point about the abscess to come up with a surgical plan? Um, I went to the OR with an ultrasound that showed a fluid collection and a kid that told me he had an indurated area, um, with the suspicion of him having Crohn's. I did not go to the OR with an MRI. My pelvis, which is what I often do with kids who've had either long-standing disease, maybe multiple fistulas or fissures in the past, something else going on. There, there, there's some literature on this, and, and there are algorithms for this treatment. And so Doctor Velasco and myself have just published a paper on this. The article you're referring to was published in the Journal of Pediatric Surgery in September 2021. What were your key findings? First, a patient that comes to the ER with a perianal lesion. Often in the adult world, the perianal lesion, the resident goes down, does an incision and drainage, and packs it and it goes away. We looked at this, we looked at over 1100 patients that came to our emergency room with either an abscess, a fistula, or a fissure. And found that male over the age of 10, and if they actually have a if you find a fistula. So those three things sort of have a much higher incidence of Crohn's disease being diagnosed. The other thing we found is that in pediatric patients, unlike adult patients, the first presenting factor for Crohn's disease can often be perianal disease. And we started to talk about the combination of perianal and ileal disease, which may be present, but a lot of patients often present with perianal disease cause that's what really hurts the child. Then you get a deeper history and go further back and you're like, oh, they do have some GI symptoms, some diarrhea, some abdominal pain, but that really didn't bring them to the emergency. Are you suggesting, Jason, that those patients who present with a perianal problem. Um, who have a high incidence of potentially finding Crohn's ought to have a more significant workup than just some I&D, or, and what, and what would that be? Great, great. Um, way to segue into what our original question was, is, is, did we need to do more of an imaging workup in this patient? And so, my answer to it, if we are not delaying treatment, The acute treatment is this abscess and this painful perianal lesion. If you can get an MRE or an MRI in the pelvis, so you can make sure there's no hidden or horseshoe fistulas or deeper lesions, and I usually try to coordinate with my GI colleagues to get the scopes done at the same time, limit the anesthesia if possible, then I coordinate. That within a 24 or so hour period and give the kid antibiotics and get the prep done. If that's not possible for many reasons and we all work in busy hospitals, then that, that, that workup might have to be um segmented, treat the perianal disease, and then work up the IBD afterwards, especially if you have a non-healing lesion. Very good, very, very helpful summary. Let's hear about the findings in the operating room. In fact, if you're listening on the state current app, you can pull up the photo of the exam that Doctor Velasquez is going to describe. When we got to the operating room, we saw the multiple skin tags. We were able to see that there were fissures that were not initially noted. On internal exam, there was an actual uh draining, abscess cavity was draining at the dentate line. So we elected to not make a counter incision to try and prevent fistula tract forming with the skin. This abscess, um, as soon as I pushed on his bottom to try to find it, spontaneously drained into his at his anus. And there was an opening at the dentate line that was at least 1 centimeter wide, and I. And it came, um, to the kind of subcutaneous skin, maybe 1 centimeter in. But I didn't feel like I wanted to make a counterincision onto his perianal skin and leave him with a drain, having this been like kind of the first thing that time I've ever seen him. So I, when I go to the OR I typically say, I don't like to make holes when there aren't holes there. I'm typically referring that to the rectum or anus and the mucosal lining, not the skin side, because the thing that's going to heal first is the mucosa. And so I, I, I'd be worried if we're gonna treat this kid with a biologic, and you're gonna heal the mucosa and you still have this abscess, you're going to get a recurrent abscess, and I think my GI colleagues would be much happier with a Seton in place or a drain in place from the infection standpoint of when they give a biologic and immunocompromise the patient. The purpose of the paper I alluded to before was, is that I don't like when we get a kid in the ER, a 10 year old boy. With a perianal abscess, and we go and make a big cruciate incision and pack it with tons of gauze, and, and that thing doesn't heal, you're in trouble. You, you may have bought that kid a colostomy or ileostomy. I would have done a taken a little 1n blade, made a stab, drained it, put a seaton in. I use a a maxi vessel, a large vessel loop as my seaton, and um we don't use cutting seatons anymore, and then continued the workup for inflammatory bowel disease. So when you're in the OR and you're struggling to find one or more openings of the fistula, do you have any tips or tricks? That you can try to help you figure it out. The biggest thing is being careful not to make a fistula where there wasn't one. And, you know, you think, aha, I found one and it's really, you've poked through into the mucosa. Then other things you can use are like hydrogen peroxide, squirting that into the tract or where you think the fistula tract is. I don't use methylene blue. Jason had methylene. I have, but I, I much prefer hydrogen peroxide. It's neater. I, the trick for me is I, I get 3% hydrogen peroxide, put it in a syringe, attach like a 20 gauge or so, 16 gauge angiocah depending on the size of the skin lesion. Uh, I usually put a speculum in the anus so I could see inside the anus, approximating where I think the fistula would be located, and then I inject the hydrogen peroxide to try to lead me towards the fistula. I feel more comfortable telling the families when I go in and I don't come out with the san that I did all these things, and the mucosa probably healed, and hopefully it'll heal from the inside out. And again, like Christine alluded to, I do not like poking holes where there isn't a hole, so I use a, a uh Probe, gently pass it through. It has a, a fish eye on the end so that I could pass aceton through if I need to. And um if I don't find a hole and I do my hydrogen peroxide uh test and it's negative, then I don't put aceton in at that time. Let's continue on to the post-op hospital course. After we finished in the operating room, we continued the antibiotics and gastroenterology saw the patient, and he underwent a bowel prep and EGD and colonoscopy. If you're in the state current app, look at the next images to visualize the findings being described. In the terminal ileum, there was significant erythema and friability indicative of terminal ileitis. What are, what are your take home points? What, what do you want our audience to know? So I think in addition to the peri, just the perianal abscess, he had these, you know, skin tags that were worrisome from the get-go, even if he had not already been being. Worked up. And then in the exam in the operating room, seeing the skin tags, the fissures, and then this, uh, abscess collection, I think was worrisome. And then, you know, upon further talking with the patient, he's had diarrhea for a year. And actually, over a 4-year period, he had actually gone from the fifty-fourth percentile. Down to the 17th percentile, definitely had fallen off of his growth curve, which was more worrisome than just a simple uh a perianal abscess that could be IND'd and sent home. OK, so you go to the OR, you drain the abscess, that's an infection. There might be associated cellulitis. Do you have a protocol of when do you start immunosuppressing the patient with steroids and a Biologic. Um, we don't have a protocol, but it's a very good point. You definitely want to get rid of the source of the infection prior and like, make sure systemically he's doing OK. Cause remember, he came in with fevers. So he was started on antibiotics. He didn't start any sort of systemic therapy until this kind of process resolved. And, for us, when we have a patient with an abscess and we're not sure if it's adequately drained, we would reimage before. Giving the biologics, or what about the steroids? The steroids probably as well, um, we would re-image, so if your abscesses or your infection is not source controlled, we would wait and make sure that is under control before giving a um immunosuppressant. What I wanna ask you guys is, maybe this patient develops another abscess, you have to put a seaton in, it has a Seton. When do you take it out? I counsel all of my patients that get Setons that they're probably gonna have it for at least 6 months. I think it takes a while for that, um, inflammatory tract to turn into a non-inflammatory tract, and it's sort of a negotiation in a sense. So I'd like to bring them back to clinic and see what they look like on a pretty regular interval to make sure things are OK. Certainly, I've had kids who've had Setons and then get abscesses other places. too, and then that, that puts them down the line of getting another MRI and trying to figure out what's going on and making sure their biologics are working. But if it's just a straight up single Cton, uh, that their other symptoms are getting better, their bottom looks good on my clinical exam in the office. OK, I take the Seton out. They continue on their biologic, and I don't see them again for bottom problem. Not everybody do I get re-imaging on. Before I take the Seton out, it's the kids that have been recalcitrant. So I've taken the seaton out after 6 months, they've been doing well. All of their numbers look good. Their fetal coprotectin is like more in the normal range. They look, they're eating well, they're healthy, their bottoms look good. I take it out, and then they start draining again, or end up with an abscess in the area that needs another ceton. Before I take that one out, I'm definitely gonna image those kids. So. I think the literature shows about a 10% response rate even without biologics, if you put a keton in and take it out, and then, um, certainly much better with biologics, so. I have a few rules that the bottom has to look better, the drainage has to be better, the pain, the symptoms have, you have to be symptom free. I make sure that they get a steady state of biologic in them, which usually means a loading dose and at least 3 more doses of medication, which usually takes you somewhere between 2 and 3 months from start of treatment. So I typically leave my seatons in for at least that period of time. On the recurrent patients, I'm much more Uh, hesitant to take it out and much more, um, cautious. I also check inflammatory markers before pulling it out, making sure the systemic disease is under control. So I look at fecal calprotectin, ESR sed rate, uh, CRP. With regard to the biologics, in, in this case, and similar cases, What exactly are you treating? Are you treating the perianal problem? Are you treating the terminal ileum? Are you treating both? And does the biologic choice or dosing matter? I think Remicade is the one that um has the most kind of literature on in terms of healing period. Anal disease, you know, and anti-TNF is, and like Humira also good. It has less literature. Um, the Stelara and like the veallizumab sometimes are used to try to treat it. Um, there's less information on Teds. And it allows for one minute of history and the New England Journal paper written, I believe it was either. 1998 or 1999, the initial Remicade paper was on perianal disease and demonstrated improved healing time, improved length of time between recurrence, um, when a combination of stan and infliximab is used versus either separately. Uh, so, our initial papers came out for perianal fistulizing diseases. Now, two decades later, it's been shown to have a tremendous amount of effects on stricturing disease and um on ileal disease. And other intestinal manifestations. I think our dosing is more, more known now. The um issue with antibodies to the biologics is known and, and being able to get levels and testing that um are all important. That wraps up our case this week we reviewed perianal Crohn's disease and a case of a patient presenting to the ED with an abscess. We discussed that males, teenagers, and patients with a fistula exam have a higher risk of an underlying IBD diagnosis and how to work them up preoperatively with lab work and imaging. We highlighted that IBD is a multidisciplinary disease and close work between the surgeon and the gastroenterologist, especially for procedures, is best for patients. We shared strategies in the OR for IND and CTO placement, then discussed the post-op management with how to follow up patients in clinic, who needs repeat imaging, and when to start biologics, as well as some other associated risks. And now, the moment you've been waiting for, our joke of the week. First, I'd like to thank our guests, Lisa and Christine for joining us as it was a pleasure to catch up with you guys and hear your case and your thoughts on perianal Crohn's disease. And we, I talked a little bit about the dinosaur ages. What dinosaur had the best vocabulary? This one's terrible, really like maybe hits the bottom of the bottom of the barrel. The thesaurus, uh, by the way, uh, that's very good, but I will tell you in follow up to that, what's the, what's the name of that dinosaur, the, the, the flying, um, the flying one, the bird, like pterodactyl, yes, you never know, you can never know when they go to the bathroom. You never know. You know why you don't know? Yeah, the pee is silent. That's phenomenal. What I love how we put it all together, man, this is just as bad as it gets. I'm, if you're still listening, thank you, but God bless you because wow. Thank you for tuning in to this week's episode of the Colorectal quiz. I welcome you to browse our previous podcast on the Stay Current app. Till next time, I'm Shimon Jacobs from Children's National. It's been a pleasure learning together with you because knowledge should be free.
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