Hirschsprung Disease - Imperforate Anus - Rectal Prolapse: 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 problems, I'll do a biopsy. Can you determine stretch sphincter. A paulus 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 patches anus, uh, Hirschberg's anus should be a normal appearing anus with a normal anal canal. Is a lot of interest in MRI for anal rectal malformations. Do you think it's of any utility in working up 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 Hirschprung's disease you're dissecting right through in the sphincter and in the anal canal and you're not placing the anus, so I don't, 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, wait, you had a question. Yeah, Jason, quick question I've asked. This question to several uh 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, that 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. 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 a 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 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, anoderm to, yeah, anoderm to the top of the columns. If that number's always there, then you skin. I mean, use a thing that everyone can, there's a true landmark, a true landmark. I, 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 the. I, I define it 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, do you, uh, 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 tip. All right, Joe, what else you got? We'll keep going because this is supposed to be fast, yeah, we're we're an hour behind. So, yeah, yeah. So let's go to the next topic. OK, OK, so my next topic is anorectal malformations again, a very broad topic we presented and Doctor Dickey helped me in this. Is a patient of hers. OK, perfect. So this is a newborn female, um, no issues with delivery, um, basically a normal echo, small PFO, normal renal ultrasound, normal sacrum, no tethered cord. That's important information. And here's a, and you're called to the NICU to examine the patient's perineum because it looks abnormal. Uh, is there any Way to change. What you wanna do? I wanna change it, but this isn't working. So this is a picture of the patient's perineum. You can see there's a catheter within a fistula type opening within the vestibule and no other anal openings are present. So my question is, what is your next step for this management for this patient, a primary repair, colostomy, dilations, or just leave it and let's see how she grows up? I start with dilations, Mark, what, yeah, dilations to start. And how about what, what age would you consider repair for these patients? What I've noticed is maybe not this exact lesion, but ones that tend to be very close to the bottom, uh, the vestibule, especially if there's something that you see in the perineum that looks anal, is that the what looks horrible initially. And a couple months or even actually in a few weeks you've noticed a difference that there is, um, uh, it's not as widely the the distance from where you expect the anus to be is not that great uh as as as in the initial examination. So I think you have to give them at least a month to see what happens before you do anything else, um, but I'm not, as long as the child is stooling, I'm not in any hurry to do this. Yeah, so at least at our shop, this is a, this is managed by a variety of ways. Several folks dilate, uh, several do the primary operation and, and a couple do the colostomies. So I, I'm always interested in this, this discussion from a, from an expert on, you know, what we as regular practicing pediatric surgeons should do because the experts can probably do. The, uh, primary nast, excuse me, primary operation in the relative newborn period, but I'm not sure that the routine pediatric surgeon should be doing that. Belinda, you want to comment? So once again. I think it depends on your comfort level. I mean, a primary repair in a newborn is, um, not the easiest thing in the world, and it's hard to tell where the sphincter should be and the dissection between the vagina and the rectum is much thinner. So if you dilations in that case is very reasonable. Um, we had talked about this the other day, you know, in, in a situation where, um, we have the luxury of TPN, IV fluids, all that sort of stuff. If you don't have the luxury of that and you need your anoplasty to heal well, then maybe a colostomy at that point in time and a repair afterwards is reasonable for those people who aren't, um, in, in the United States, North America, um, where they have that. I also feel, do you, what are you, what are your feelings on if you do dilations for a long period of time, do you think that causes some inflammation and some I think it causes scarring and inflammation. So I usually only go up to a 7 or 8 and put them on stool softeners. I've had some people come who've dilated up to 11 or 12 and. It, it's, it's not fun to repair at that point. That was a softball, but that, that was my point is I think you have to be very careful about the dilations and causing inflammation because your your dissection at age 3 months or 6 months can be just as tedious if you're causing local trauma I agree with that. So moving on a little further, hey Jason, just one other quick question. So I was always taught that you wanted, uh, you did not want to do the dilations because the meconium is sterile, and if you're going to do the operation, you ought to do it in the newborn period as opposed to dilating and doing it at 2 or 3 months of age when you've got stool, which is not sterile. And so if, if you're gonna wait for 2 or 3 months, you ought to do a colostomy to divert the stool. Is that a. Is that an old fashioned concept or where do you stand on that concept? I'll just call it a concept. I'm not sure if it's old fashioned, but, um, I think we are, we are very conservative in our treatment of, uh, anorectal malformations, especially in the postoperative care and feeding and trying to what I call a medical colostomy for the period of a week or so by making them NPO and not feeding them and giving them hyper alimentation. Um, but there is no data to support that. I know there are a number of pediatric surgeons that will repair an anorectal malformation at whatever age with dirty stool and feed the child the day 1 or 2 days post-op when they recover from the anesthesia, and I'm sure that incidence of complication is probably similar. So I don't think we, I think we more, we don't do the operation in the immediate newborn period, more for it's a difficult dissection. And Belinda and I have been discussing, I think it's harder to tell exactly where the center of the sphincter is in a 2 kg baby than in an 8 or 9 kg baby. So because it's my fault, I'm being told we are way, way over. So let's just do rapid fire questions without discussion for the rest of this. Just perfect answers, OK. Last question on this topic. You see this picture that's up on the screen, if you call it, and if you can't see, it's a vestibular fistula and there's no other hole, meaning an absent vagina, which is a situation you can get into. Um, in a patient that if you don't get a good perineal exam preoperatively, what should we do? That's my question. No, that's just, just go ahead and tell if in a patient, in a patient that has good prognosis for bowel control, you'll have to do either abort the operation and come back another day with a definitive plan, but if you have a plan in place where you think you could use a, a um. A graft, whether it be a piece of colon or a piece of small bowel, whatever you like to replace the vagina, and then bring your, your rectum down, uh, your fistula down as you pull through. If it's a patient that has a poor prognosis for bowel control, patient with. Agenesis or tether cord or or other reasons why they may have poor bowel control, then you could use your rectum or your fistula as your as your vagina and then just take a more proximal piece of colon and bring it down as you pull through. Short answer. That's, that's good. I'm gonna skip um the rest of this and just for the sake of time, uh, that's a vaginal septum, but move to my other topic which is another no controversy involved. So my last discussion that I was asked to speak about was on rectal prolapse, tremendous amount of literature on this in the pediatric population. Um, I'm kidding, we, we have room to improve here, um. So first thing when you're dealing with rectal prolapse is to discern whether it's full thickness prolapse as seen on the pictures, ah, the two pictures on top of each other, or a partial thickness prolapse or mucosal prolapse is seen. Uh, under the wording and so this is the patient could be of any age, um, but I wanted to ask the question of what workup do you perform for patients with rectal prolapse? Do you scope the patient rigid or flexible? Do you do a contrast enema? Do you do anal physiologic testing which could include manometry, um, EMG, etc. or, or do you do a combination of testing and we could go back to that later and and. in the talk, the other question I wanted to ask the audience was, do you test for CF? I was trained to test for CF. I answered the question on my boards to check for CF. I've never picked up a patient with CF in my practice, and reading the literature, neither has many other people picked up patients. You might have a CF patient that presents to you with rectal prolapse, but have you picked up a case of CF? So I think that'll be interesting to learn. But the million dollar question is next. If medical management is not successful, so I think many of us would treat the patient for constipation, sit, have him only sit on the toilet for 5 minutes, and, but you, you went through that process and you went through it for 6 months, 1 year, 2 years, and it's still going on. What operation would you perform? And I, I didn't include laparoscopy versus not, but just would you do a recopexy, a recopexy, and resection. Uh, a DeLorme, an Altmeyer inject sclerosing agents or others as there's newer gold standard operations in the adult literature right now, uh, mostly a ventral rectopexy, which I don't think has hit the pediatric literature too much yet. Rapid fire and does anyone do any so I do, I do a resection and a rip stain recopexy. OK. Anyone do anything else different? OK. OK. Anyone else? David, I haven't done many of these, but I've done, uh, I haven't done many, but I've done a few anal circles, uh, which actually works well, especially in the small babies. Um, I get achondroscend on if there's a huge redundant segment resect, but definitely laparoscopic rectopexy to promontory. So the litter injection of sclerotic agent, you've injected sclerotic agents in fellowship at least. I, I haven't had, I haven't had to do it yet, but that's actually it was filled because out of peanut oil and I haven't done that. What's that hypertonic saline. I can tell you when it recurs to re-operate on those patients is not going to matter at all. Yeah, a lot of people, I'm just looking at this, a lot of people did the sclerosing agent. I, I have not had success with, I can't think of a patient that I didn't end up doing a rectopexy on. So the literature and some of it's, most of it is in the adult literature, but I'll share with you, even in the pediatric literature, it sounds like the transabdominal approaches are have less of a recurrence rate whether you do a resection and rectopexy or recopexy alone, but going through the abdomen has a About a 5% recurrence rate versus somewhere between 15 and 20% with the transanal approaches, whether you're doing a transanal pull through or a um an Altmeyer type procedure, um, has a higher recurrence rate, at least in the literature. The literature also states that because I used to do a resection and ectopexy, so if you're having problems controlling the constipation, that might be a better procedure, but there's a higher. Um, complication rate with patients who are having a resection, obviously a resection and a and a Pepy versus just epy alone. There's literature now where patients go home the next day after a rectopexy or even same day surgery, so that seems to be the the theme. The only other thing I wanted to talk about, and that's demonstrated in this cartoon here, um, which is a newer. Procedure in the in the literature in the adult literature and really popularized by the group in at the Cleveland Clinic and a few other centers is the ventral mesh rectopexy, and I think many of us are afraid to use mesh and there's definitely reports of erosion in the population, but I have one patient where I've operated on twice through the abdomen and then the. The operation was this ventral mesh rectopexy, which seemed to have solved the problem, at least for now, and it's basically putting a piece of mesh on the anterior surface of the rectum, bringing the rectum up, not dissecting posteriorly, and then tacking the mesh to the sacral promontory is the basic description of the procedure, and you can do that open or laparoscopically. So the idea is that change some of the angulation, change some angulation, and the literature, if, if you want to go into the pathophysiology of rectal prolapse, especially in the younger patient, they think the angle of the rectum to the anal canal is more of a straight shot when you're younger and it becomes more of an angle as you get older and so this is sort of trying to change that, although this is the procedure used in many. Women, you know, many adult, older adult patients, primarily women who have, uh, pelvic floor relaxation issues. So I think those are the things that are up to date in rectal prolapse. There's no perfect operation. If you know of one, let me know. That was great. That was a good summary.