The Colorectal Quiz Episode 20: Hirschsprung Disease Obstruction Part 2

Hey there listeners, it's Amanda Jensen from Riley Children's. Last week, we left off talking about Hirschwrung's disease and the obstructed patient, and it was part one of a two-part series. For this week, uh, we are gonna pick up where we left off and uh talk about this patient. So if you remember, this was a seven year old who presented to the ER with a history of Hirschprung's disease and a previous pull-through, and he presented with obstructive symptoms. We initially treated him for Hirschprung's associated enterocolitis, which included IV antibiotics and colonic irrigation, which he did not tolerate unless he was under anesthesia. So with an obstructed Hirschprung's patient, we last left off discussing the contrast. enema. So this showed a narrowing of the most distal 6 centimeters of his colon and a dilated proximal colon. And we most likely thought that this was an anatomical or pathological issue of retained ganglionosis or the swabbe cuff restricting the most distal part of the bowel, although we did not know what type of pull through he had previously. All right, I first want to reintroduce Doctor Jason Fisher from Cincinnati Children's. Doctor Fisher, why don't you start us off? When you go to take a patient with obstructive symptoms from Hirschprung's disease, what's your process? Do you have a checklist of things you do when you go to the OR to do an exam under anesthesia for these Hirschprung's patients? All right, Hira, why don't you start us off with the discussion? Right. So, for, obviously for any, regardless of the type of pull through, we always have to think about if they have a stricture. So, to do a good exam under anesthesia, we, I usually do a digital exam and then feel for the anastomosis and if there's any circumferential stricture. That's Doctor Hira Ahmad from Seattle Children's. Some advocate for actually using a Hagar dilator to actually size the, you know, opening to make sure that it's of adequate size. The other thing to do is pass a Foley catheter to make sure that there's no twist in the Poulter segment. Sometimes if you're blessed with longer fingers, you may be able to feel that and enter the peritoneum, but I have size 6 gloves, so I usually use the Foley catheter to determine if there's a twist or not. And then for wave cuff, you'll again feel this 1 centimeter or 2 circumferential narrowing. That's not quite the anastomosis. And then for the pathological issues, thing we need to rule out for it is with full thickness rectal biopsy. For the patients who've had a previous Duomel, we wanna make sure that we actually do their full thickness rectal biopsy posteriorly, um, because that's the segment that has the, that should be ganglionic. So, the two things I do want to mention, you mentioned Duhamel. That's Doctor Mark Levitt from Children's National. When you do your rectal exam, you want to try to feel for two lumens and see if there's a spur between the two lumens. Because that can be problematic. The stool flows down, does not exit the anus, flows up into the Duomel pouch. The Duomel pouch fills with stool and compresses the ganglionated pull-through. And a, a fix for that is to take out the common wall. Occasionally, the Duhamel pouch itself needs to be excised, and the other thing I want to include in this conversation. is before the patient goes off to sleep, it's important to look at the anus and see if it looks like a good sphincteric contraction or patulus. Obviously, a pattuus patient, patulus anus is not gonna get enterocolitis, but it's just to complete the discussion about the examination under anesthesia. You wanna be sure that the sphincters are OK and not, not, have not been overstretched. And when you do your biopsy and your exam, you're going to take a very close look to ensure that the dentate line has been preserved at the original pull-through. I will tell you, if the dentate line has been lost at the original pull-through, or the sphincters have been overstretched, the one thing you're not gonna have in this patient is enterocolitis, which is quite sad. You will have fecal incontinence, right? So it's rare in this type of patient that you'd find that, but it's important, and we can go into a whole discussion of theories behind how that happens and why you don't have enterocolitis in those patients, because I think both Mark and I have been working on projects of using Botox to prevent enterocolitis with the theory of sort of Chemo denervating the nerves in that area instead of having permanent stretch slash anatomic destruction from surgery. 01 thing I was gonna add is make sure to actually use the Lone Star retractor. That's Doctor Rebecca Rentia from Children's Mercy in Kansas City. Because you really wanna make Make sure that you get a circumferential view of the area. It's, it, a lot of these things sound like you could just kind of do it or get a little bit of a look or take a speculum or something, but you really wanna set up so that you can look at all these things carefully. Excellent. So this is what we look for on. Our exam under anesthesia, I know our, many institutions have pre-made operative reports to sort of make sure that you touch on all these subjects, you know, it's sort of a checklist model. But Rebecca, tell us what you found. You, you, we had this contrast study, you then went to the operating room to do, do an exam under anesthesia. What did you find? Yup, so what we found was we found an intact dentate line without stricture, a normal size anus, you know, good sphincter tone even under anesthesia, some liquid stool that expelled on digital examination. We found no swabbe cuff, so there was no rubber band or constrictive feel on the outside of the pull-through. And so we didn't feel any of that actual structuring or narrowing on digital examination that correlated with the imaging. And so our next step in all of this, the mucosa itself looked very healthy, and so we decided to do a full thickness biopsy. OK, so let me just, uh, Mark, Mark taught me this many years ago, sadly, cause it's just been many years. Um, because learning to feel for the suave cuff is not, I think, something that is in the textbook or is. You know, on, on your first time going to the OR doing an EUA something you think about. But what I learned is, I think, first of all, you have your roadmap from your contrast study, knowing where you think this might be happening if it's a problem. And I do on digital exam, so I take my finger and, and hook it, and try to feel it, see if I get that hook or that muscular cuff outside. The pull-through bowel. Remember, in this case, we didn't know what surgical procedure was done, so we, we're uncertain, but I use my uh digital exam and my finger to sort of feel and hook that muscular cuff, and I feel, if I feel that, And I see those images on a contrast study, I, I get concerned that the cuff might be constricting, and you get sort of, sort of what we see, see on this contrast study of that hourglass appearance sometimes on contrast study and imaging. This, now in hindsight, looking at this contrast study, that narrowing went all the way down this. And there was no hourglass shape. It was more of a total imagination or narrowing all the way distally. Yeah, this, this hard to tell. The view of that contrast study, I agree, could either be an obstructing cuff because there is a presacral space, but it could also be a distal ganglionosis. I think it's important to discuss the repeat biopsy after a pull-through. This is something my, in my own thinking that has evolved. I think if you rebiopsy a patient who's doing poorly, and you find no ganglion cells, It's very likely that that is an a ganglionic or a transition zone pull-through. Recognize, of course, that you have to be aware of the possible sampling error, and if the patient has significant obstructive symptoms, that's probably a patient that needs a redo. The uh more common finding is ganglion cells present with hypertrophic nerves. And this is a little bit more controversial. I will tell you, I have done redos for those patients in problematic patients who are very obstructive behaving. But I don't know if the original pathology was off, or something happened over time that made the pull-through decompensate, leading to the hypertrophic nerves, because we do know that hypertrophic nerves in the presence of ganglion cells can occur in functional constipation patients, and maybe a Hirschprung's patient who's not emptying well, Let's say, cause their sphincters are not relaxing, might develop obstructive symptoms, and might develop hypertrophic nerves, so I can't blame the original pathology, but I will tell you, if I have a patient with significant obstructive symptoms, who has a biopsy, and the biopsy shows ganglion cells and hypertrophic nerves, I have offered to redoing those patients, particularly. If that's, and, and I'm talking if that's the only indication, in this case, there might be a cuff as well, and those patients have done well. But I do want to recognize that until you compare the two pathologies, you don't know. The pathology originally might be perfect, and the pathology today might be problematic, and something evolved, or the original pathology perhaps didn't assess for hypertrophic nerves, and in fact, the surgeon left the patient with a, With a transition zone pull through. I'm gonna throw out something that maybe will blow your mind, or maybe you'll just call me crazy, probably the latter, but I'm gonna throw it out there anyway. I always am troubled by when we do these pull-throughs on these patients, and they're a week or two old, and you get your biopsy back, and you bring them to the OR and you do your pull-through in this 10 day old neonate, and we leave. 0.5 centimeter, 1 centimeter, 1.5 centimeter, you, you do your dissection about 0.5 to 1.5 centimeters above the dentate line, do your anastomosis in this 10 day old baby, and now you have a 7-year-old whose bowel has significantly increased in size over that time. So that, let's just for easy math, say, it was a 1 centimeter, what I'd call rectal cuff that you left behind before bringing your pull-through down. Is now, let's say 3 centimeters, or 4, or 5. Well, is that the problem that this kid did well until that cuff got to a length, that's a ganglionic, and now it's causing obstructive problems. And you do your biopsy, 2 centimeters above the dentate line, and it's not really the pull-through that's the problem. We left too long of a cuff at that time. Yeah, so my big question for you, for you, what do you mean we? Because I don't leave cuffs, because I think cuffs are problems, and that's why we do a, I do a Swenson, and even the suave is out there, the devotees to the suave, yeah, but you start your, your Swenson dissection 0.5 centimeter, 1 centimeter above the dente line. Yeah, but there, but there's no, oh, you're saying that the little, it's not a cuff like a slave cuff, it's, it's your distal rectum, your native rectum that you've left behind. I have not. I didn't understand, sorry. I, I have not had that problem. I think a good pull-through can overcome that little bit of a ganglionosis, and you're leaving that gangliosis behind intentionally, so you don't come too close to the dentate line. What I was saying though is for the suaveist out there, the suave is approaching a Swenson in most of those people's hands, and many of them have actually gone to a Swenson too. And so, a mini, mini cuff is basically what, I, I quote, Dan von Almen, who used to call it a swabson, um, which is one of my favorite, um, uh, descriptions, because it's basically a 1 centimeter cuff, but in the original laparoscopic suave that Keith Jorgeson talked about, he recommended a 5 centimeter cuff, and I think that's way too long, if you look at the original paper. Remember, the Swenson was the first operation, and it is the purest of the operations, it leaves the least amount of Hirschprung's behind. If you do a swabbe, and the reason why the swabbe and the Duhamel were developed is because surgeons were doing the Swenson in too wide of a plane. They were injuring the nerve errientes that is in the, in the mesorrectum, and if you stay right on the bowel wall, you don't get into this trouble, but they didn't know that at the time, and they blamed the procedure, developing then the suave and the Duhamel. But the suave is, has its, it saves that plane for sure. But then there's the cuff, and then you need to cut that cuff if you're gonna do a swathe, and I think what happens is sometimes the cuff fuses back together, or you don't cut it all the way, so it rolls up a little bit and makes a, a ganglionic obstructive ring around, around the pull-through, and that's what I suspect is happening here, and you can feel it on rectal exam. And um um Jason, you described how I like to do that. It's very important to put your finger against the sacrum and pull down. And you feel this rubbery tissue that's, that's outside of the pull-through, that is the cuff. And I have a feeling that this is the problem here, or this is a, um, a transition zone pull-through. So maybe we can get to the, the, get to the answer here and Rebecca can uh do the big reveal. How are we gonna do the reveal? It's gonna be like a balloon and, and, and, and Betty comes out, but, uh, what, how are you gonna do this? I, I, I think we need to edit in that sound where we have the fireworks going off. You mean this one? What do you think this is a professional operation? I'm in my garage. So the big reveal here, uh, Doctor Levitt described it perfectly earlier. This was a transition zone from where we did a really, uh, nice generous full thickness rectal biopsy. So, no ganglion cells and nerves up to 80 microns on average. All right. So, so there you go. So, did you feel a cuff at all on the posterior aspect of this? It was hard to, um, I did, but it was hard. It was not, um, it was not extremely obvious. And so to me, what was more correlate correlatory to this picture at the time was that it was a transition zone. I didn't feel like a very firm ring around the area. All right, so this is a, a ganglionic pull through. It explains what's wrong, and I think this patient's gonna need a redo, and the approach for that, I would do a total body prep, and then I would go transanal in prone position first. I think the redos are really easier prone, and you go as far as you can dissecting the distal pull-through. There is a remote possibility you could get that healthy bowel down to reach. But if not, you need to be prepared to go into the abdomen, either with laparoscopy or with laparotomy if needed. So, you wanna tell us what you did here? Yeah, so the special nuance about this child was that the child could not be irrigated. So, unfortunately for the child's safety, they had to be diverted, and then they underwent a pull through that was completed transanally. We did see, um, quite a significant amount of suave cuff at the time, and there was a transition zone, and we got to normal ganglion cells, or a large amount of ganglion cells, normal nerves. So the child awaits takedown of an ileostomy. Yeah, I think that's a really, really safe, good way to do it. So, I'm very impressed. Where'd you get your training? Uh, somebody named Doctor Levitt. I don't remember. That was an awfully long time ago. Never heard of him. It's an awfully long time ago. So, um, what else were we, were any, anything else we missed, Jason, that we should cover here in this, in this case? I, I think we did a good job. I think, I think the points to remember are initial treatment, rectal irrigation. It's a must, and it should be done early and Hopefully every institution has a protocol so that when you see this patient, you react quickly and efficiently. 2, I think, a systematic workup, which Rebecca showed us beautifully in this case of first resuscitating the child, then getting some imaging studies, which helps lead to the diagnosis, and a rectal exam under anesthesia with all the steps that Hira adequate, uh nicely told us about to make sure that you don't miss anything when doing your Exam under anesthesia, and then once you put it all together, you figure out what the treatment is, and in this case, it was retained a ganglionosis or a transition zone bowel, which required a re-operation to bring down ganglionated bowel. So, I think it's a great example of what most pediatric surgeons will see in their clinic at some point during their career. So, awesome case. Yeah, and just to reiterate, you gotta know the anatomy. Gastroenterologists gotta know the anatomy. Was it a suave? Was it a Swenson? Was it a Duhamel? Re-biopsy. If, by the way, none of those things are wrong, it could be the sphincters. We've talked about Botox at another, at a different, um, a different podcast. Yes, you also cannot forget those sphincters. All right, and that's a wrap. So just to recap, in our obstructed Hirschsprung's disease patient, as Doctor Fisher mentioned, we wanna make sure to number one, resuscitate the patient, IV antibiotics, irrigation. If the child is unable to undergo adequate. Irrigations at the bedside, bring them to the operating room and do irrigations there. And then, if unable to evacuate that stool, as Doctor Rentia mentioned, she ended up doing an ileostomy for that child until she could figure out what was mechanically or anatomically wrong with the child. And then, in figuring out what is the exact problem, doing a barium enema, as we discussed, and then a rectal exam under anesthesia. So, the rectal exam under anesthesia. As, uh, Doctor Frischer and both, and Doctor Levitt discussed, doing a really good rectal exam, feeling for that cuff, using your finger to kind of pull down and feel if you feel that wave cuff. And then taking biopsies and figuring out if it's a transition zone or not. And then based on all of those findings, making your operative plan. And now for our colorectal joke of the day. I was listening to um a great comedian the other day, uh, Zach Galifianakis, who I'm sure you guys know of, uh, and he actually made a colorectal joke. You know what he said? I have no idea, but doesn't everyone make colorectal jokes? Yeah, they do. We are, we are, um, a good, good source, um, in a good, uh, location for joking, but he, he said the following, he said, basically, the only good time to shout out loud in public, I have diarrhea. Is when you're playing Scrabble. Uh, yeah, it's probably not good to shout that out that often. I wonder how many points that would be. Well, I actually thought about it and it's too many letters, so, uh, no, that's not true. You can add it to someone else's letters, so you can figure out a way. And if you get triple word score, you're in good shape. All right, and we are out of time. That's a wrap for episode 20, Hirschsprung's Disease and the Obstructive Patient Part 2. Until next time, this is Amanda Jensen at Riley Children's, and remember, knowledge should be free.