Hirschsprung Disease: Update Course 2015

All right, we're gonna move on now from the chest to the colon and uh Doctor Jason Frischer, who is, uh, the head of the colorectal Center at Cincinnati Children's Hospital, he's gonna talk to us about Hirschprung's, and these are gonna be rapid fire. So 5 minutes on Hirschprung's, 5 minutes on imperfor anus, and 5 minutes on rectal prolapse. I recall having a full day of uh Hirschprung's webcast, and now I get 5 minutes. You get 5 minutes. Take the whole day, put it into 5 minutes. Basically, what are the key questions? 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 um suction rectal biopsy that demonstrates no ganglion cells, hypertrophic nerves, and abnormal ACE staining. So you have confirmation of Hirschrung'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? Uh, I'd do a laparoscopic biopsy and uh 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, I mean, 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, if you just do the transanal one to begin with, you're gonna, 1 in 10 times or so or 1 in 15 times, sometime in your career, you're gonna find one that's higher than you wanted it or a total colon, and then you're gonna 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 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 three stage type procedure to do that. So I, 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, 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 gonna 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 trans anal 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 fit, 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 I mean what's so because I could do it from the bottom in the same time frame if it's a nice standard rectosigmoid it'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 transanally 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.