# Hirschsprung Disease Rapid Fire: Update Course 2015 — GCMD Library

Dr. Jason Frischer of Cincinnati Children's Hospital Medical Center, presents on Hirschsprung disease. Dr. Frischer discusses Hirschsprung disease and its complications.

Type: video · 12 min · posted 2019-01-11
Canonical: https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985

## Chapters
- [0:00](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=0) Case presentation and operative approach poll
- [3:44](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=224) Invasiveness of transanal versus laparoscopic approach
- [5:40](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=340) Complications workup algorithm
- [7:59](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=479) Dentate line definition and dissection level

## Statements
- "For the average pediatric surgeon doing transanal pull-through without prior biopsy, approximately 1 in 10 to 1 in 15 cases will have aganglionosis higher than expected or involve total colon" — Holcomb (clinical) [1:47](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=107)
- "Putting three abdominal incisions may be less invasive than prolonged transanal dissection with torquing in the anal canal" (opinion) [3:50](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=230)
- "Laparoscopic mobilization with three 3-millimeter ports can be completed in approximately 45 minutes" — Jason (clinical) [4:57](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=297)
- "Standard rectosigmoid Hirschsprung disease 6 to 10 centimeters up can be done transanally in approximately 2 hours" — Jason (clinical) [5:06](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=306)
- "Post-Hirschsprung complications divide into obstructive symptoms (enterocolitis, failure to thrive, abdominal distention) and soiling issues (true incontinence versus pseudo-incontinence)" — Jason (clinical) [5:53](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=353)
- "Obstructive symptoms after Hirschsprung surgery require determining whether the cause is anatomic or pathologic" — Jason (clinical) [6:13](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=373)
- "True fecal incontinence after Hirschsprung surgery can result from injury to the sphincter or injury to the dentate line" — Jason (clinical) [6:20](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=380)
- "Workup for post-Hirschsprung problems includes water-soluble contrast enema and exam under anesthesia looking for dentate line position, stricture, stretched sphincter, and twist" — Jason (clinical) [6:53](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=413)
- "If no anatomic cause is found on exam under anesthesia, biopsy should be performed" — Jason (clinical) [6:53](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=413)
- "A stretched sphincter is determined by observation of a patulous anus, whereas Hirschsprung anus should appear normal with a normal anal canal" — Jason (clinical) [7:20](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=440)
- "MRI utility for Hirschsprung complications is uncertain because unlike anorectal malformations where anus placement within sphincters is assessed, Hirschsprung dissection goes through the sphincter and anal canal without anus repositioning" — Jason (opinion) [7:33](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=453)
- "Starting dissection approximately 1 centimeter above the dentate line in a newborn may result in 2.5 to 3 centimeters of retained aganglionic segment when the child reaches 7 years old" — Jason (clinical) [8:33](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=513)
- "If dissection starts too high and subsequent biopsy is not taken high enough, the biopsy may show transition zone rather than definitive aganglionosis" — Jason (clinical) [8:59](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=539)
- "Injury to the dentate line can render patients fecally incontinent, which is a devastating injury" — Jason (clinical) [9:11](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=551)
- "The dentate line is defined as the transition from squamous epithelium to columnar epithelium, located somewhere within the anal columns" — Jason (clinical) [9:37](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=577)
- "In J-pouch surgery for ulcerative colitis or FAP, dissection is performed right at the top of the columns or slightly lower if polyps are present in that region" — Jason (clinical) [10:07](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=607)
- "Ultra-short segment aganglionosis can be overcome with laxatives, whereas fecal incontinence cannot be overcome" — Belinda (clinical) [11:50](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=710)
- "The surgical approach hedges on the side of leaving ultra-short segment Hirschsprung disease rather than injuring the anal canal" — Jason (opinion) [12:04](https://library.globalcastmd.com/watch/hirschsprung-disease-rapid-fire-update-course-2015-985?t=724)

## Transcript
All right, excellent. So I have a couple of patient presentations to do. The first one's a simple just to get a poll of the audience. So a newborn with increased abdominal distention, not tolerating feeds and hasn't passed meconium, you get a contrast study with whatever material you want to use, and it's demonstrated on the screen. What, how would you proceed? Would you proceed with a laparoscopic and you get a suction rectal biopsy that demonstrates no ganglion cells, hypertrophic nerves, and abnormal ACE staining. So you have confirmation of Hirschprung's disease with this contrast enema. Would you do lapa laparoscopic biopsies, mobilization of the colon and a transanal technique? Would you do a transanal dissection and then only go to laparoscopy or a laparotomy if needed? Would you do an open biopsy and immobilization in the colon, or would you do a leveling colostomy or some other technique you may have developed? How about in the audience, Mark, what would you do on a with this contrast enema? I'd do a laparoscopic biopsy and pull through in laparoscopic mobilization, yeah, yeah. Mac, what same. What happens if it showed a more Standard rectosigmoid, I think this is a higher, it's almost like at the descending colon, where's the transition, but if it was a rectosigmoid lesion that you think you could reach from below, would you still do laparoscopy or would you start transanally? No, I'd still do laparoscopy because I think you can be fooled. Same. I think that Jason, you're more of an expert at colorectal disease than I am, and there are 2 or 3 others around our country, but for the standard. Average pediatric surgeon, if you just do the transanal, if you just do the transanal one to begin with, you're going to 1 in 10 times or so or 1 in 15 times, sometime in your career, you're going to find one that's higher than you wanted it or a total colon. And then you're going to have the colon in your hands and not know what to do with it. So I, I just want to put that out there. Yeah, it's not going to happen very often, but it will probably happen in your career at some point. And so if you're going to do the transanal without a biopsy, just figure out what you're going to do in that situation. And you know this more than I do, but you're an expert, and, and I'm just an average practicing pediatric surgeon. I, I, Doctor Holcomb, I appreciate your comments, and I, I agree with you. I, every time that I Do a primary trans anal, I get. I tighten up a little bit to make sure that I'm confident that I can do, and I have an exit strategy in mind if I get in trouble, um, but The safe way to do this is some sort of biopsy, whether you do it laparoscopically or a laparoscopic mobilization, maybe do the biopsy through the umbilicus if you want to get a nice full thickness biopsy, whatever your technique or trick is, that's certainly the safest way to go. Jason, um, I was noticing on the poll, almost 30% of the folks in the audience are going to do a leveling colostomy, so you can comment on that. I think Uh, I think that's a safe thing to do. It depends on what pathologists, your pathology at your home institution, if you have concern about your pathologist, if you don't have someone comfortable reading, um, for Hirschberg's disease, uh, that might be the safest thing to do. I know people who go on mission trips and trips where you don't have pathologists. It's a almost a 3 stage type procedure to do that. So our audience is diverse and so it might depend where they're coming from. Let's ask those who answered leveling colostomy if you could leave a comment on on on why you chose that. I just want to make one comment that Belinda and I talked about and uh is that I, I don't believe that a pure trans anal is necessarily less invasive. In other words, I believe that putting 3 incisions on the abdomen is actually less invasive in my hands, in my hands, certainly not in your guys' hands, but in my hands, I'm going to be torquing in that anal canal much more than if I had done it laparoscopically and freed everything up from the abdominal approach. I think this is just like anything else. Belinda, go ahead. It depends on your comfort level. I mean, we've talked about this before, you know, I, with the transanal trying to get high past the pelvic reflection. You're, you're pulling and you're stretching. So if you're comfortable with that, then OK. But um, I think if it's beyond that, then you're going to do laparoscopy or laparotomy if you're not Belinda and I talk about this a lot, and it's about a comfort level and how I take, I watch the clock how long I'm doing a trans anal dissection because I do not want to be stretching on those sphincters for 4 hours trying to dig up in a dark hole when I could put a scope in. I easily put 3. 3 millimeter ports in and mobilize laparoscopically in 45 minutes or so and get the same what's so because I could do it from the bottom in the same time frame if it's a nice standard rectosigmoid's rectosigmoid you can get it done in a couple hours. I mean if you know where your level is and if you have a good contrast study, I think the image on the screen I would do with laparoscopy without question, but a standard rectosigmoid 68, 10 centimeters up, I could do transamally in the same time frame. OK. We, did we cut you off? Were you gonna say something? OK. So that was not controversial at all, and I'm gonna, that was the simple question. Now I wanted to go on to complications a little bit, and this is the, the patient population I really enjoy taking care of, and I think that pediatric surgeons need to be aggressive at caring for these patients because they are our patients, and I think we know what's. How to help them best and so patients who have complications after Hirschberg's disease, we sort of put into two piles. They either have obstructive symptoms and things like enterocolitis, failure to thrive, abdominal distention, or they have soiling issues and then divide them into true incontinence and pseudo incontinence. And then the patients with obstructive symptoms you have to discern whether it's an anatomic problem or a pathologic problem. And Soiling, like I said, you have to discern whether it's true fecal incontinence, and that could be due to injury to the sphincter, injury to the dentate line, or pseudo incontinence. Is there a constipation issue causing the incontinence? This is a, I include this in the slides, and I'm not going to go over it. I think Jack Langer published this in a paper, um, a few years ago, this algorithm, which really does a nice job of describing how to work up patients with, um. With problems after Hirschrung's disease. The workup I, I include, includes a contrast enema, water soluble, and then an exam under anesthesia, looking for the listed items below which dentate lines, stricture, stretched sphincter, um, looking for twists, and then I, if I don't find a reason for the patient having problems, I'll do a biopsy. A paulous anus. So if it, if it, so just looking at it, not, no. The question was how do I determine a stretched sphincter, and it was basically by observation. If you have a patchless anus, Hirschberg's anus should be a normal appearing anus with a normal anal canal. Is MRI for anal rectal malformations. Do you think it's of any utility in looking at problems? I think with anal rectal malformations they're looking at placement of the anus within the sphincters and you're splitting the sphincters when you do that procedure versus Hirschberg's disease you're dissecting right through in the sphincter and in the anal canal and you're not placing the anus, so I, I'm not sure we haven't done it. I don't know if other centers are doing anything of that nature. So patient two is a 3 year old. Uh, you had a question. Yeah, Jason, quick question I've asked. This question to several experts and I'm interested in your feeling. What do you, where do you start your anal rectal dissection? And if you use the so-called dentate line, how do you define what the dentate line actually is? Oh, great question, and I took those slides out because this is rapid fire, but from to answer, where do I go above the dentate line? I go approximately 1 centimeter above the dentate line, so some people will go 0, like 0.5 centimeter, 1 centimeter, 1 centimeter, 2, and I, I worry about this because if we're doing this in a newborn, that, that distance of 1 centimeter might become 2.5 or 3 centimeters when they're 7 years old and having constipation issues. So then you're left with a situation of what some would call short segment or ultra short segment Hirschprung's disease. And then if you go back and biopsy, you, if you don't go high enough, you might get a biopsy that shows transition zone or something, so it's a great question. I go approximately 1 centimeter above the dentate line. I want to ensure, because, and maybe we have a biased population in what we see, that I do not injure the dentate line because the patients who are rendered potentially fecally incontinent due to injury to the dentate line is a devastating injury for those patients. I'd go 1 centimeter or slightly less above the top of the anal columns. What do you call the dentate line in relation to the columns? So you go 1 centimeter above the columns. So I, I measure the dentate line is the transition of of columnar epithelium. And so I look at, and I obviously don't have a microscope there, but it's somewhere within those columns where that is. So I take. I go at the line of where you see the transition from squamous epithelium to columnar epithelium and go 1 centimeter above that, so it's easier for me just to see, to use the columns as a landmark. Is that right or not going pretty high. I do that when I do my J pouches. I take my, when I do my J pouches, I'll go right at the top of the columns in an ulcerative colitis patient, an FAP patient. I may even hedge a little lower than that, especially if they have polyps in that region. So, so how, how variable is, how variable is that distance of the from the skin to the top of the columns if it's almost always exactly the same. But it's not, and it grows with the patient, but they're almost always doing it right neonate as a neonates. Yeah, I'm talking about a newborn, a newborn in a newborn, if you measure it, you guys should do that since you get so many is measure if that is almost always the same in every newborn, to measure from, from skin to the top of the columns, annoderm to, yeah, anoderm to the top of the columns. If that number is always there, then you skin. I mean, use a thing that everyone can, there's a true landmark, a true landmark. I give this presentation in our course that we have in a couple of weeks, and I have pictures from the internet from Netter and from other sources. The dentate line, whether in cartoon fashion or in anatomic dissection, is pointed to, and you could call it the pectinate line, the dentate line. It's very variable where people point it at. It's somewhere within those columns where people point at you as the surgeon. everybody always talks about, I define as the transition from squamous to columnar, and that's where the bottom of the columns really sort of lie. I don't know, Belinda, Keith George and I used to argue about this. So I also have taken the tops of the column as a standard spot, and whether you go there, you go above there, I have found that to be the best landmark. I think we actually go to the top of the columns. I mean, when we do it, because you always hide the dentate line and go above, and, and we probably leave a zone of angliosis, but we do that on purpose because you can overcome that with laxatives. You can't overcome fecal incontinence, right? So I, I think we hedge on the side of leaving an ultra short segment Hirschrung's disease versus injuring the anal canal. Great, that's a great.

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Not medical advice · citation policy: https://library.globalcastmd.com/ai