Hirschsprung Disease Part I with Marc Levitt

Today's podcast is on Hirschsprung's disease. But before we get into Hirschsprung's, here's a review of last month's podcast on appendicitis with Witt Holcomb and listener comments. Last month on Stay Current. Tell me about your antibiotic regimen. We have found that ceftriaxone and metronidazole is the most cost-effective uh antibiotic duo. What about antibiotics for non-perforated disease? If it's non-perforated disease as identified in the operating room, then the patients do not get another dose of antibiotics. How long do you keep your non-perforated patients in the hospital after surgery? Uh, in the last year or so, we have been sending our non-perforated appendix patients home the same day. Can patients with acute appendicitis be treated non-operatively? And as a general statement, I would say 2/3 of those patients are treated without the need for an operation and about 1/3 require an operation. The title of this uh podcast is, is called Stay Current, and I am absolutely not Current after what you just described. So I want to dig a little more deep into that. All right, well, now it's time for the listener mailbox, and this is where we go through comments or questions from listeners relating to the last podcast, and that one was on appendicitis with Doctor Witt Holcomb. And with us we have my current research fellow, Doctor Nick Bruns, who is a general surgery resident at the Cleveland Clinic. Nick, what do we have in the mailbox? Well, thank you, Todd. One comment came from Dr. Farid El Alahi from the Tripoli Medical Center. His first question was, what is the negative appendectomy rate? All right, what did you find? Well, the best data actually comes from a paper by Kurt Newman in 2003 in the Journal of Pediatric Surgery. What did it show? It reviewed the PIS database and determined that the negative appendectomy rate among hospitals was a median of 2.6%, but there was a pretty significant range anywhere from 0 to 17%. So there's some pretty impressive variations. So that's about the number I tell people, about 3% a negative appendectomy rate. So what else, what else was out there? Any other questions? Yeah, well, the same surgeon actually asked about the management of an incidental Meckel's diverticulum during laparoscopy. And what did you find out about that? Well, there's data either way. There's a nice review of this by Zany in 2008 in the Annals of Surgery, and they recommended leaving the Meckels, stating that the risk of complications is just not worth the extremely low mortality rate which they cite to be 0.001%. On the other hand, there's the 50 year Mayo Clinic Experience that was published in 2005 in the Annals of Surgery, and Dr. Park was the first author. So that recommended a selective approach by resecting any meckles with any of the four features, so it was age less than 50, male sex, length greater than 2 centimeters, or the presence of histologically abnormal tissue. So in the pediatric patient, whether you choose to resect or not, there's data to support you. What do you do? Well, if it looks like a problematic meals, like it's really long or thick, I'll take it out by stapling across the base, but otherwise I'll leave him alone. Did you ask Doctor Holcomb what he would do? Yeah, so Dr. Holcomb doesn't routinely take out meckles if he finds it incidentally on exploration in any age. That's correct. So I guess this doesn't help because we both have different opinions on this. So I guess you can do either approach. So any other questions in the mailbox? Yeah, so we had another comment from Dr. Gavin Falk, a fellow at Miami Children's. So he commented that there's data on same day discharge for non-perforated appendicitis. What did you find? So he actually cited a paper from Miami Children's that was published in JAMA Surgery in 2012. Al Khoury was the first author. So I looked at around 200 consecutive patients with non-perforated appendicitis who had an appendectomy or interval appendectomy. 80% of those were same day discharges, and they found that both groups, the same day discharge versus overnight stay, had similar complication and readmission rates. So how do we interpret this data? What does this tell us we should be doing? Well, I think this is encouraging for hospitals considering creating a protocol for same day discharge. All right, Nick, thanks so much. Let's head on to Hirschprung's disease. Stay Current is an audio publication designed to keep healthcare professionals up to date with standards of care and new emerging ideas. These brief interactive podcasts are designed to keep healthcare professionals current while on their commute. Each episode will be hosted by key opinion leaders in the field. Stay Current is produced and edited by Nicholas Bruns, Mark Schwachter, Todd Ponsky, guest editors, Witt Holcomb, Dan von Almen, and Jay Grossfeld. Welcome to Stay Current in Pediatric Surgery. I'm Todd Ponsky, a pediatric surgeon at Akron Children's Hospital, and today we're going to be focusing on Hirschprung's disease. And with us today we have definitely an expert in the field, Dr. Mark Levitt, who's at Nationwide Children's Hospital. Mark, thank you for joining us today. Hi, Todd. It's great to be here. Mark, before we get started, can you give everyone a, a, a quick synopsis of, of where you are and what goes on down there at Nationwide? Sure. Well, I'm at Nationwide Children's Hospital, Columbus, Ohio, United States, um, very center of the state of Ohio in the Midwest. I'm a pediatric surgeon. I'm the surgical director of our colorectal and pelvic reconstruction Center, which is a center focused on the collaboration between Pediatric surgery, pediatric neurology, pediatric gynecology, and GI motility for all problems that could possibly relate to the colon and pelvis. Perfect. How long have you been there, uh, in Columbus? We're about to celebrate our first year anniversary. Perfect. And I know that, uh, you've been having some fun down there and things have been going well, and we appreciate you taking the time to do this with us. So, Mark, we're gonna have some fun here and we're gonna go into the first patient. So let's start off with a newborn who has Hirschsprung's disease. So Mark, you, uh, you get called by the NICU that there's a 4 day old full term in the NICU who's been having some feeding intolerance, and they've noticed some abdominal distention, and then they noticed one episode of bilious vomiting. You go down and see the, the patient, and, uh, before you start asking questions, just as you walk in, you notice that the patient has a pretty impressively distended abdomen. How do you evaluate these patients with these types of evidence of a possible distal obstruction and, and what's your usual method for working this up? Um, so Todd, this is a very common scenario for pediatric surgeons to deal with. There are many medical explanations for it. Then, of course, there's the um more surgical-related things that pediatric surgeons know about, and those are the distal obstructions. You know, in general, we always get a plain abdominal X-ray. And in general, we tend to evaluate such a scenario with a contrast study. Although, of course, with Bia's vomiting, we need to do a limited upper GI and make absolutely certain that there's no malrotation, and then proceed with a contrast study from below. So when you take the baby, you get your contrast enema. And there is suspicion for Hirschprung's disease. There is a, what looks to be a transition zone around the rectal sigmoid junction. What is your next step on this child? So if you have a contrast study that looks suspicious for Hirschsprung's, I think it's important to sort of be working up the patient, but also recognize that you need to treat the patient. So one of the, probably the best treatment for Hirschsprung's disease is irrigations. You know, it is very rare that Hirschberg's disease is a surgical emergency. But if you don't irrigate and overcome the distal obstruction, it will become an emergency. Um, and I can't emphasize enough how important it is to know how to do a proper irrigation. Can you go through that, uh, exactly how you should, we should, we should be doing these irrigations? Yeah, so if you take a large bore tube, like a 20 French Foley, and get some warm saline irrigation, I take a 60 cc non-morlock syringe. And I basically um insert about 10 to 20 cc aloquats at a time, passing it with the lubricant, of course, instilling saline, moving the tube to and fro, advancing it as far as you can without needing resistance, and then you take the 60 cc syringe off the tube and let the fluid mixed with stool drip back. And as long as you're putting in fluid and getting fluid back, you can keep irrigating. You should feel like you're getting meconium out, you're getting the tepid, um, pond kind of fluid out, and the baby's abdomen should become less distended. And then you have had a successful irrigation. And if that works, we tend to do that 2 or 3 times a day, even for several days. So Mark, uh, just to clarify, You take your 60 cc syringe through that 12 French tube, and by the way, I, I use actually 20. I would use a 20 French. That's what I was going to say. I thought you said 12, so that's perfect because we use an 18 French, but I like 20 even better, um, large, large catheter. That's, I think one of the biggest mistakes I've seen. is when I end up showing up to see what they've been using, that's too small of a caliber tube. You then said you inject 2010 to 20 at a time. Do you inject 10 to 20 at a time, let that drain out, and then put in the next 10 to 20? OK, that's correct. And I think the other mistake people make besides using too small of a tube is they just put the fluid in and they let it sit there. Well, that's not an irrigation. That's called an enema. That's right. Babies, babies with Hirschberg's disease have no ability to expel the enema fluid. And they just, uh, you know, will walk away after 100 ccs have been instilled and you haven't accomplished anything. So do you find yourself sort of manipulating the tube, sliding in and out, slightly, massaging on the abdomen, or does that not work? Yes, I, I absolutely would do that. You keep moving the tube into various positions. Mark, you, you have this baby and you've been doing these irrigations. Let's say the baby. Ah, ah, for whatever reason, you, you, you don't want to operate on them right now. Let's say maybe they're really small, they're premature, or for whatever reason. Do you ever send them home with irrigations, or do you keep them in the hospital? I am not a fan of sending babies home on irrigations, um, because, uh, it's not as reliable as doing the irrigation in the hospital and then operating. If you have a particularly responsible family, this, that is not an unreasonable option. OK, perfect. Mark, what if you don't have a pediatric pathologist at your hospital? Can you just do the pull-through based on the contrast enema? Yeah, I mean, I think you have to confirm the diagnosis pathologically. And if you are somewhere in the world, and I think we'll probably get to this discussion, but if you're somewhere in the world without pediatric pathology, and you need to intervene on a baby that's sick, not responding to irrigation, and you do not have pathology, then you need to divert that baby. My personal recommendation is to divert that baby in the ileum. So tell me how you do your biopsies. I take one sample. Um, as long as you're in deep enough, it's important to remember that you need to be at least 1 centimeter in from the dentate line. If you biopsy too close to the dentate line, everyone has, um, an a ganglionic segment there, and you could get the wrong answer. Um, and someone thinks you have, the baby has Hirschberg's disease when they actually don't. You have to be careful not to biopsy too high. If the gun gets sent in too, too deep and biopsies at 4 or 5 centimeters, which I have seen happen, you may miss a lower zone of Hirschberg's disease. So can you go through and explain then what you want to see on these biopsy results? And the pathologist really needs to be prepared for evaluation of Hirschberg's disease, and you as the surgeon cannot accept a pathologist's report unless there is the absence of ganglion cells and the presence of hypertrophic nerves. The absence of ganglion cells alone is not Hirschberg's disease. That could be a biopsy that's taken too low. What about using acetylcholinesterase? Um, that is a reasonable adjunct. It's not, um, absolutely vital and it's not done at all centers. Um, a lot of pathologists like it cause it's a confirmatory, but the standard of care that you must have is an, uh, just traditional H&E of, um, uh, whether they're ganglion cells or not and whether the nerves are hypertrophic. That's usually enough, and that's pretty much what we do here. OK, so this is the, the same baby now. The irrigations are not working. The distention persists. What do you do at this point? You cannot have that baby remain distended because distention is stasis, and stasis is the problem with Hirschberg's disease. Interestingly, if you put a cork in a newborn baby and they didn't stool for one week. They would not get sick. They would just be distended and have hard stool. But Hirschprung's disease has an immune component to it, and the, the lining of the bowel, the mucosa, is much more susceptible to translocation. Why this is, no one really understands it. There's a lot of work being done on enterocolitis, but the bottom line is stasis in a Hirschprung's patient. Leads to bacterial translocation and a very sick baby from bacteremia. What you're describing is a patient who is not responding to irrigations. And the only way that could be the case is if the tube isn't reaching to the ganglionic segment that's dilated. So that could be a higher type of Hirschrun's, usually left colon or transverse colon or right colon or total colonic Hirschrun's disease. And if irrigations are not going well, and the baby is ill, that's luckily very rare, you need to do something. And that's when the discussion about whether to do a stoma comes up. And then there's discussion about going to the operating room and bringing out the dilated segment, which is the commonly done move in the developing world, which is a very reasonable and very safe thing to do even without pathology, cause the dilated segment is going to be ganglionic. And even if it's not perfect, meaning it's transition zone, in the form of a stoma, it will work well, usually, because a stoma is a low-pressure system and it should just empty. Now, if you have pathology that's willing to help you at 3 o'clock in the morning and you can do frozen section, you could do what's traditionally called a leveling colostomy and pick the spot where the ganglion cells are good, and then ultimately, that would be the spot for the pull-through. But I can tell you that I personally have changed my practice over the last few years. And I don't do leveling colostomies anymore. You can be misled by an inaccurate frozen section result, particularly if the transition zone is higher. It's much harder to make an accurate frozen section call as you move higher in the colon. So, I have actually started to do biopsies in a sick baby that needs to go to the OR and get diverted. I have done permanent biopsies, but a diversion at the at an ileostomy and wait for permanent section, which is much more accurate, and then at some point down the road, do your pull-through. When do you time the operation and how do you prep them? I just find the soonest selective time to schedule that case and in the next several days. No, I don't do any go lightly prep or anything like that. I just make sure they're irrigated well from below. OK, now let's talk about the different operations now. So we have Swenson and Suave and Duhamel. Can you go over some of these and sort of your thoughts on, on these different, uh, surgeons? So let's talk about Swenson. You know, the first operation that was invented was the Swenson by Orvar Swenson, and he did a transabdominal operation where he entered the abdomen and dissected down full thickness down to below the peritoneal reflection, and then pulled through colon. And it was a beautiful operation. Um, well, that operation, I believe, was done often incorrectly with a full thickness, but more than full thickness, meaning the perirectal dissection, the deep pelvic dissection of the rectum, was done too wide. And this is something that was already known to be a problem in the adult world because those patients, if that dissection of the rectum for adult reasons was done too wide, patients were left with fecal incontinence, urinary incontinence, sexual problems, impotence, etc. So to respond to that, two very brilliant ideas came up. One was from Dr. Suave. Um, uh, in Italy, and one was from Dr. Duhamel from France. And what Suave did was he through the abdomen again, and I think it's important to recognize that these original operations were done transabdominally through the abdomen, um. The, the dissection was with, was inside the outer wall of that rectum. It was quite brilliant. So to keep the outer wall intact so you can't injure it, um, Suve came in a little bit tighter and essentially did a mucosal dissection. Um, and, uh, that was quite brilliant, and that sort of became the standard and the original suave actually. Once the pull-through was done, um, Dr. Suave would pull through the colon and actually leave it coming through the kid's anus for a week, would go back to the operating room and then do the colo coloanal anastomosis. It was actually Dr. Scott Bole who said, Well, why don't we just do this all in one stage? So the correct terminology would be a suave-bole operation with the bole modification. Duhamel around the same time. Came up with another brilliant idea, and that is to leave the original rectum, remove the ganglionic portion from just above or at the peritoneal reflection, and the ganglionic portion gets pulled in a retrorectal position. And then connected so the two lumens are mated into one lumen by a stapler, also a transabdominal operation. So those became the three operations, and Swenson really took a back seat because people thought it was fraught with complications. The next, the next moment in history, I would also add, and American surgeons don't know a lot about the Rabine procedure, but that is a very commonly described operation in Europe. Which is essentially a very low anterior resection where the rectum was left behind, but the ganglionic bowels has made it to the rectum with approximately a 4 to 6 centimeter original rectum sitting there that's a ganglionic. It's fascinating actually that, you know, of those four procedures, the only one that actually leaves behind virtually no Hirschprungs is the Swenson. The suave leaves behind the outer rectal wall. The Duhamel leaves behind the original rectum, and the ravine leaves behind the original rectum as well. Amazingly, many, many of those patients did perfectly well, and I think that has to do with the fact that the ganglionic bowel, if it's good, can overcome a lot. As we'll talk about when we talk about the problem Hirschrung's patients, sometimes the ganglionic bowel is wimpy and not very successful at overcoming any obstruction at all, and that's when patients get into trouble. I think the next aspect of history was to do the entire operation as a, as a primary operation, essentially with no, with no initial, with no initial colostomy. And you know, I would give Henrys credit for that who was in the Philippines. I actually got to know him when I worked some time in New York, and he would do a leveling colostomy in the dilated portion, and the babies would never come back to his clinic because there was such a social stigma in the Philippines against having a colostomy. So out of desperation he just went ahead and did the entire operation that I just described primarily, and it worked. Um, the next aspect of history that's I think it's important, and I'm skipping through, you know, decades of history. This is very well described actually. There's a very nice, very nice article written by Dr. Grossfeld on the history of Hirschsprung's disease, which I would strongly recommend people read. And was was to apply a minimally invasive approach to avoid the laparotomy component, which has been essentially what laparoscopy has done for the world is to avoid big incisions. And Keith Jorgeson was the one who said, I have laparoscopic skills. Let's apply it to Hirschberg's disease. And what he did was he delineated the colon and did all the colonic work. Elegantly laparoscopically with a suave dissection from above, then the concept became, and of course from below with some transanal work, but the concept then became this transanal, and the transanal, which everyone now talks about. It was an absolutely brilliant idea that Luis de la Torre and Jack Langer came up with during the same period of time, and it was starting the dissection, intending to do a primary operation and starting the dissection from below. I will tell you, this is history that, you know, no one really knows and probably doesn't need anyone to know, but when in my time working with Dr. Pena, we were very into the full thickness rectal dissection because that was the same dissection one used for a standard PAP which Dr. Pena obviously invented. And we said, well, why don't we do the same for Hirschprung's disease? And I still remember that we did a couple posterior sagittal incisions to mobilize the rectum through a posterior sagittal incision, full thickness, and we were very proud of ourselves until the dilatorre and Langer idea came out and we said, Why the hell did we make a posterior sagittal incision? Because you can do everything that you need to do transanal. So the transanal now, and then of course we adapted that technique, but then the discussion is, well, what plane do you use? So if you approach the patient transanally for the dissection, you can do the suave and essentially begin a submucosal dissection before you break into all of the planes, and that's probably the most common operation that's done. OK, so that was a great review of all the different technical options available for a Hirschsprung's pull-through. But Mark, what do you do in your practice? More recently, um, I have started to do about 10 years ago, I would say a transanal Swenson. So when you say Swenson, you have to remember that was transabdominal. When you say swab, that was transabdominal. So I think it's appropriate to say. Transanal suave-like or suave plain or transanal Swenson-like or Swenson plane, and I mean by that full thickness. And that's my preferred approach because I think it is the purest of the operations. It leaves behind no Hirschsprungs except for the very, very bottom just above the dentate line. And if you find the right plane, it's quite elegant and bloodless. If you find the wrong plane, you can really injure the patient because then you're too wide. As, as, as one has known and, and uh that we talked about before. Dr. Swenson himself, who recently died at the age of, I believe, 105, was a big fan of helping us, help reminding us of how good the Swenson was, because he said, you know, I've been saying the Swenson operation has been good all along. People just weren't doing it right, and it got a bad rap, unfortunately. And so he was very thankful that we were re-promoting the Swenson, and a number of centers now have taken that on and have enjoyed doing the Swenson. I think it's a cleaner, neater operation. The Suave is absolutely beautiful, of course, and what you'll notice actually is people are making the suave more Swenson-like over time. Even the suave enthusiasts are making shorter and shorter cuffs. Dr. Jorgeson's original description of the laparoscopic wave, which essentially was the laparoscopic pull-through. Recommended a 5 centimeter cuff. Nowadays, Langer and Del Tre would do a 1 centimeter, maybe 1.5 centimeter cuff, essentially a Swenson, so one could call it perhaps a Swanson, um, but, um, basically the concept is to protect the nerves, but I think you can do a very elegant Swenson plane dissection. And get very good results, and we've shown that without leading to incontinence or impotence or any urinary problems. Mark, that was a great overview of all the different techniques. Is there a place that you can think off the top of your head of where people might be able to go and watch demonstrations of these techniques? Are any videos yet available online or for purchase? I'm, I'm happy to share my video of how to do the transanal Swensen type dissection. Um, anyone's welcome to email me and I will send it to them. It's M A Rc. Levitt, L E V I T T at nationwide Children's.org anytime. Perfect. Thank you, Mark. Let's talk about, you know, there's a lot of controversy with the laparoscopic approach. That's the approach that I prefer. What are your thoughts on that? I know there's a lot of controversy. Well, I mean, laparoscopy is a fantastic adjunct to Hirschprung's disease. I think the controversy really becomes, do you apply laparoscopy to all Hirschprung's patients, or are there somewhere in which it's appropriate to do transanal only. Um, and, uh, my feeling about this has, has, has definitely changed because I've seen a lot of morbidity that has resulted from an overly aggressive transanal-only approach, um, trying to reach the transition zone with the, with the valiant attempt of never going into the abdomen laparoscopically or via laparotomy, and I think that that is a problem. Uh, to be honest, so if, in my view, if you have a very reachable transition zone comfortably, sort of mid-sigmoid, and by the way, you, it's obvious, the transition zone, so many babies, it's not obvious, but if it's very obvious, which happens from time to time, and it's mid-sigmoid and you can comfortably reach that transanally, it's a beautiful operation where you don't go into the abdomen at all laparoscopically or open. The entire operation is done transanal. Um, I prefer doing it prone. I'll do a total body prep and turn the patient prone. I'm much more comfortable prone because of PARPs, and I think anyone who tries a transanal approach prone will never go back to supine because the tough part of the dissection is the anterior part, and it's nice to be looking down on the harder part of the dissection. But I will only do that if I'm very, very confident that the location of the transition zone, and I feel it's reachable transanally. Anything else. I will do laparoscopy first and make sure I know where the level of transition is. For those out there who do Duhamels, obviously you don't want to do a transanal and burn the Duhamel bridge, and those are folks who usually would do a Duhamel if there was a total colona Hirschprung situation. Um, and those patients are at risk for. Having burned your desired bridge of leaving the rectum in position, I personally do ileoanals in total colonic Hirschprung, so for me it doesn't burn a bridge. However, for those who want to do Duhamels for ileal Hirschprungs, then they don't want to do a transanal at all. Although I will tell you that you should pretty much, pretty much be able to figure out if you're dealing with a total colonic patient. If you have a very typical Hirschprung's presentation. A pretty obvious looking transition zone, a patient who's responded well to irrigations, that's not going to be a total colonic case. Total colonic patients are, they sort of limp along. The diagnosis isn't made right away. The contrast study is not typical, and the irrigations aren't going well. Those are patients that need a laparoscopy first and find the biopsy, find the biopsy location. So I think that's the controversy. I mean, if, if I, if I was talking to a fellow. And they had to take their boards. I would tell them you do laparoscopy in all cases and find your level. Don't do a transanal only because people will tell you, well, that, you know, the contrast study showed a transition zone, but actually it was wrong. It was much higher than that, etc. But I will definitely do transanal only in the appropriate case, you know, Mark, the globalcast events we do are meant for debate and discussion and putting the gloves on. I don't want to do too much of that here, but I will tell you that. I do think that they should all be done laparoscopically. I am not convinced that laparoscopy is more invasive than pure trans anal. There's, it's just so easy and elegant to get the entire dissection done without pulling on the on the rectum. So I do them all that way. Well, I would, I would agree with you, and I particularly am swayed by the fact that if you do a trans anal and you're too aggressive in your attempt to visualize the dissection. You would have been much better off with a lower laparoscopic dissection, no question about it. Having said that, if it's reachable and I feel that I can do the transanal without that stretching, and I think if you do the transanal right, particularly in the Swenson plane, the entire dissection is sort of At the anal level, it's not in too deep. You're you're really doing a beautiful operation. So, but most cases I think it's appropriate to put in a laparoscope and check and do your biopsy and then use the laparoscope to do a variety of things. The laparoscopy is great for taking down the splenic flexure if that's necessary. The laparoscopy is great for taking the vessels, taking the IMA, and preserving the the marginal arcade that goes down the left colon and sigmoid. And the laparoscopy is particularly excellent for the deep pelvic dissection. And if you do all that well, your transanal is quite minimal that you need to do. So let's talk about some tips and tricks for preserving the countenance mechanism. What do people do wrong? What are some of the pitfalls that you can advise against here? So I think the biggest problem is people don't give themselves good exposure and then they start the dissection too low and they injure the dentate line or resect the dentate line. Or they give themselves very aggressive exposure and they overstretch the sphincters. I think those are the two biggest morbidities in the technique. That's a great pearl mark. So up until now you've given us a great review of the history of Hirschsprung's disease, the workup and diagnosis of Hirschsprung's disease, but I think what people really want to get from you is how you do your technique. Can you take us through the steps of your technique and give us some tips and tricks on how to do an effective operation? So I like to use the Lone Star retractor, which is the, which are those pins that give you a circumferential display of the anal canal, and I'm very methodical about that. I put that in the skin first. I expose and visualize the entire circle of the dentate line. Then I will one by one replace the pin deeper, deeper past the dentate line so that the dentate line rolls underneath the pin. So you have a beautiful circle that's visualized and you cannot see dentate line. Then I will place a purple mark um and then we put the, put the stitches, the circumferential. I put 50 silk stitches in that purple line, and that purple line. Delineates 1 centimeter proximal to the dentate line. So you have preserved the anal canal and the dentate line plus an additional 1 centimeter of columnar epithelium before you've started your dissection. And in that regard you have not hurt or put yourself in a position to hurt the anal canal, then you don't want to overstretch those pins because then you don't, you don't want to hurt the sphincter. Mark, what you have repeatedly pointed out is that surgeons often start the dissection too close to the dentate line and that it is critical to start the dissection 1 centimeter. Deep to the dentate line, is it a problem leaving that 1 centimeter of Hirschprung's ganglionic bowel? By definition, you're leaving behind 1 centimeter of columnar epithelium, that of course is Hirschberg's disease, but the ganglionic bowel is able to overcome that. And of course you're leaving behind the smooth muscle, the internal sphincter, which has a problem by definition with relaxation. However, that can be overcome by good ganglionated bowel pushing through and eventually the baby recognizing how to relax that sphincter over time. So that to me is the purest operation is then to start that dissection Swenson, but you could just start that dissection right then and there as a suave. But either way you must preserve the anal canal and the dentate line. And if of course you're doing a Duhamel, none of what I said is really relevant because the entire rectum stays preserved. You just are going to pull through the ganglionic bowel in the retrorectal position. OK, so now you've marked out 1 centimeter proximal to the dentate line. Now how do you start your dissection? So once I have that exposure, I've marked my purple line. I've put 50 silk circumferentially, and I will start a dissection with electrical cautery. I like to use a very fine needle tip cautery, and I go straight for the Swenson plane. It's, it's full thickness. There's a nice areolar plane that you find, and once you get good at it, you will love that plane. You, you recognize that plane right away. It's avascular. And then you begin your dissection. You're pulling the rectum forward and you're finding those bands, and it's a very similar dissection to the PAP technique. When you mobilize a rectum, you want to get inside that whitish fascia, and as you continue to mobilize, the rectum frees up. And remember, the rectum doesn't really have a mesentery. The rectum's blood supply is intramural. So as you get higher and you reach the peritoneal reflection, Um, that's when you start to see some sigmoidal vessels where rectum transitions to sigmoid. The other thing I like to do, and again, we're in a prone position, is I've recognized that the anterior rectum frees up much quicker than the posterior rectum, and I take advantage of that fact. So I will break into the peritoneal reflection, um. Anteriorly first. Now I don't know if we're talking about a situation where we're doing transanal only or we've done the laparoscopy, but let's say we've done the laparoscopy already. We've made our dissection. You break through circumferentially and you pull the colon out and you're ready to go with your anastomosis. If you're doing a transanal only, when you break through the peritoneal fraction anteriorly, often you can pull the sigmoid out. And you can actually do a biopsy of that sigmoid, full thickness. And then shove it back in and while the pathologist is spending is spending their time analyzing that specimen over the next 40 to 45 minutes, you continue your posterior rectal dissection. And then if you time it well, right when you're done with freeing up the entire rectum, the pathologist calls and says you have a good location, and then you can do a case like that transanally in an hour and a half. It's very nice when that happens. It doesn't always work out that way, of course, but very nice when that happens. Of course, if you're doing it laparoscopically, you already have the biopsy off and you sort of know what you're dealing with, and then the transanal is just a matter of getting the colon out. Mark, for those who prefer to do the laparoscopic approach, can you describe how you do your laparoscopic biopsies? So If you're starting with laparoscopy and you're doing a biopsy, what I like to do is I put the laparoscope in the umbilicus and then I move the laparoscope to the high right upper quadrant. It's important that the surgeon stands by the baby's right shoulder. I think this is true for any rectosigmoid dissection, be it ARM or Hirschprung's disease. And then in the umbilicus, you can put a grasper and grab various parts of the colon, pull them out the umbilicus, and then do a full thickness biopsy and drop it back in. Now, what a lot of people do is they do a laparoscopic biopsy. So they grab the serumuscular layer and they cut, hoping not to injure the mucosa. However, there is a pitfall there, and that is that the seromuscular layer can have the, can have ganglion cells. But the submucosal layer, which was not sampled by that technique, can have hypertrophic nerves. So if you're going to do that, you may get a very good sense of where the transition zone is. But when you're ready with your pull through, send another full thickness biopsy. Our pathologists will not accept a specimen of colon that does not include submucosa. That's an interesting, and I have not done that. I I'll do my laparoscopic biopsies the way you described, and I send my specimen as a full thickness for permanent, but I don't get another frozen section once I'm going down transanally. But that's a good bit of advice, Mark, and I would, I would definitely, you know, I think it's very reasonable to do that kind of biopsy because Then you haven't violated the mucosa. You don't have to sew it up. It saves a lot of time if you do it laparoscopically, if you do it through the umbilicus, it's very easy to do it full thickness and then sew it up. But that is only going to tell you ganglion cells in the serial muscular layer. It's not going to tell you whether your submucosa is OK. So, and, and I never realized that you could have good gang ganglion cells within the serial muscular, but no ganglion cells in the submucosal layer. Well, you may have ganglion cells in the submucosal layer, but you might have hypertrophic nerves there. And therefore you then you basically have set up a transition zone pull through and you want to avoid that. So if you're going to do that technique, then I would send a full thickness biopsy later to confirm that you're happy with the level you're choosing. I love it. So Mark, really quick, so let me describe the way I like to do it laparoscopically because we do it different. So just to give another, and this is along the lines that you were describing, I put in 3 ports. I put the scope in the umbilicus, and then I put a 3 millimeter just above that and then one on the right lower abdomen, and I do, I do the laparoscopic biopsies with the scissors. It's actually a fun technique to learn to do. It's a good exercise for the trainees as well. Um, and then I stitch. I do put a stitch, you don't need to, um, but it's a nice exercise in intracorporeal tying as well. Um, question for you, do you, um, do you get a confirmatory, and I'm guessing your answer is no, do you get a confirmatory biopsy very low down before you do anything? Laparoscopically, do you go down in the perineal reflection? No, because I already have a rectal biopsy that I know it's Hirschsprung's. I'd like to try to do this operation with one biopsy. If you pick the, if you pick the right spot, and if you pick the healthy spot in the, usually this proximal sigmoid or 2 centimeters above where you see the transition. Yeah, something like that, and I want to make sure that our pathologists are not only telling us about ganglion cells but also about the quality of the nerves, and I think a subjective description of hypertrophic nerves is not enough. We like to have an actual number. I'd like to know the microns, and the microns. Be 40 microns or less. Anything bigger than 40 microns is transition zone bowel, and I go a little bit higher. And the concept of go 5 centimeters above, and I think that's inaccurate. I think transition zone is a spectrum. I've seen 10 centimeter transition zone. I've seen 3 centimeter transition zones. So you really need a confirmatory biopsy, and your pathologist can. If properly trained, gets you the ganglion cell information and also the quality of the nerves to the to the micron measurement. OK, Mark, that's actually an important point to repeat. So to confirm that you are out of the transition zone, you need your pathologist to tell you that your nerves are less than 40 microns. Is that right? Yes, 40 microns or less. I would consider normal, right? Anything above that, you're concerned that they're hypertrophic. Yes, OK. Um, and that also answered my other question. I know some people do 3 biopsies at a time and send them all, and they, so they don't waste time. I, I agree with you. I try to do as few as possible. You know, I just do 1 biopsy in the good bowel. I already know it's Hirschprung's, you know, this is sort of bringing up the point of how high to go, right? So trans anal or laparoscopically, where do you go? I mean, what if you could get away with just mid rectum, is that enough? Most of these patients, it's somewhere in the sigmoid and you bring down the proximal sigmoid to the, to your, do your coloanal anastomosis. I think trying to get transition, trying to get your anastomosis in the lower sigmoid is not ideal. Many of those patients have not had enough of a pull through. So I like to have a straight colon. I like to have the sigmoid loop out. So from a technical point of view, it's nice. You take the IMA, you preserve your arcade. The left colon and sigmoid are now nice and straight, straight down into the perineum. Makes for a very easy to irrigate baby, and I recognize that that may take a little extra of the bowel that you didn't necessarily need to take, but it's, it's, you're very confident that you're going to be in the good zone of the bowel. So I've seen a lot of patients that have not had enough of a pull through where the entire sigmoid loop is still there, and they need a redo to remove more of that. And so, so, Mark. Do you just, yes or no, do you start the dissection before you have your biopsy results back? While they're waiting to, to do the path, are you doing your laparoscopic rectal dissection? If it's a very obvious transition, yes, I, I, I would start if I'm worried at all about a higher, higher zone, you know, more, I would say honestly anything proximal to the splenic flexure. I personally would do colonic biopsies and an ileostomy and wait because frozen section has notoriously been fraught with errors in those cases. And there is no urgency. And by the way, another option is to take your biopsies and quit. Don't divert. Come back 3 or 4 days later and take the baby back to the operating room and then do your pull through. The point is that frozen section can be misleading. So if I'm very confident in transition zone, the bowel looks beautiful in the proximal sigmoid and the bowel looks leathery in the lower sigmoid and rectum. I proceed. But if I'm not happy and I don't like how the left colon looks, I will send multiple biopsies and either quit. Usually I will just divert with an ileostomy so the baby can thrive and do well quickly and wait for permanent section. If it's a classic sigmoid situation, then you do your standard pull through that uh that we've talked about, OK. Um Before we get on to. Some other situations, let me ask you, are there any other tips and tricks that you wanted to talk about regarding the technique or the setup? Yeah, I think the one thing I wanted to mention is that if you're going to do transanal only and you're in a prone position when you When you free up the anterior rectal wall, and then we talked about how, you know, then we go to the posterior waiting for the biopsy. What I like to do is I put sequential stitches. On the inferior, sort of the 6 o'clock position, of course, you're in prone position, so the 6 o'clock position of the rectum, as I pull the bowel out, I continue to put a couple stitches in the same line. That not only helps me pull the bowel out. Because I put those on mosquitoes, but also keeps the bowel aligned and makes it less likely that you're going to spin the bowel. You don't want to, you don't want to twist it. So I put in a stitch and I pull. I put another stitch and I pull, and you'll get to a point where the sigmoid just comes right out of the abdomen. And you have 3 or 4 stitches that helped you do that, and they're all at the 6 o'clock position, and it helps you from twisting the pull through. Now for me, who does this laparoscopic, so my child is supine. Do you still do that, but then at the at the 12 o'clock position? Yeah, it's a nice thing to do. And then, and then because I'm so neurotic about that detail, at the very, very end, I'll pass a tube and make absolutely certain that I didn't spin at 360 degrees. I like that. I like that. And, and by the way, for the setup for the laparoscopic, if you're supine. Uh, someone showed me a trick, I think this is also Scott Bollinger, of tying the feet to this mobile, uh, sort of ether screen type of thing that goes across the table, and you could put the feet up or down, up or down based on if you're going from below or from above, and that's a good way to do it and you put a little foam bump on. The bottom, you know, as far as laparoscopy, I don't know if we completed where all the trochars go. I personally like a in any rectosigmoid work. I like the camera in the high right upper quadrant. My left hand is the umbilicus. My right hand is the deep right lower quadrant, and then my assistant is in the high left upper quadrant to hold the sigmoid over. I think that's a great That's a great, so you actually put in 4 ports then, is that correct? Yeah, I'll use 4 ports both for a like a bladder neck fistula for ARM and a Hirschprung's because I really like holding up on the sigmoid by your your assistant who's in the left upper quadrant, and then you have a nice right and left hand that you can do your mesenteric dissection. Yeah, and I do want to point out that for both the way you and I both described it. Um, surgeon, the operating surgeon is mostly on one side of the patient, so you're, you're, you're on the, you were on the right side. Your ports are sort of on the right side of the abdomen. And a lot of people feel that that, oh, this rectum is midline. I'm going to put my left hand on the left side and my right hand on the right side. It's actually quite tricky. Would you agree? It's very uncomfortable. And I also tell the fellow to make sure to stand, make sure that they can stand comfortably at the baby's right shoulder, which means move the baby down, get the anesthesia machine out of the way. I want, I actually make them stand there before we prep so that they're so that they're not pushing themselves into the anesthesia machine after everything is set. I like these tricks I'm learning here. It's actually been helpful. The one thing, you know, that we, thanks to you recently hired Dr. Aaron Garrison from when he worked with you in Cincinnati. And he, I hope he doesn't listen because I'm going to say that he's one of the most phenomenal surgeons I've worked with, and he is actually convincing me to start moving from the Swenson, I mean from the suave to the Swenson. So listening to you describe all of this, I'm starting to become to drink the Kool-Aid and become a believer. Yeah, I hope you do, and I can tell you, once you find that plane and There's no better person than Aaron to help you find that plane because he's superb technically. Once you find that plane, you will recognize that it is much more pleasant than the suave. It's much more elegant. There's no bleeding at all, but I would strongly recommend try transanal prone because it's a really, really neat way to look at the rectum. Of course, if you're laparoscopically and you do most of your work first laparoscopically. I don't think you need to go to the effort of actually spinning the patient prone. Just lift the legs and do it because you're not doing much of a significant amount of transanal dissection. But if you're going transanal only, so you have a fair bit of dissection to do, I would suggest doing that prone. Yeah, I think that's a great, great tip. Mark, I wanted to ask you before we move on to the tough situations, what do you do with these patients postoperatively? What do your postoperative orders look like? When do you send them home? When do you bring them back to the clinic, and how often do you dilate them or irrigate them? Yeah, so I'm very conservative about the postoperative period. I find that often patients are fed too early with Hirschprung's disease, and many of them go home. I am passionately committed to not have a baby come back. And readmitted with enterocolitis, and I, what I will do is I will wait until the belly is absolutely soft and flat. They're having bowel function, passing gas, stooling. I actually will get an X-ray because I think abdominal distention is sometimes subclinical, and I don't feed that baby until the X-ray looks good and the belly's flat, and that usually takes 3 or 4 days. I know there are people that will feed those babies on day 1 or 2, and I think 9 out of 10 times that works beautifully. But if that baby eats a little bit distended, goes home, they can come back with enterocolitis, and I don't want any baby to come back with enterocolitis. So a couple extra days for me is not a big deal to wait and not take one step forward and two steps back. I make sure the families know how to do irrigation pre-op. I make sure they have all their supplies and know what an irrigation is, and they, and I make them paranoid about distention. So that they will call me, come to the clinic, and get irrigated by us if the baby is distended in the postoperative period, and then we would continue irrigations thereafter if the baby needs them, which is rare but sometimes necessary. My routine has been to at one month to check the anus in the clinic with Hagar dilators, not my finger, and then dilate, and most babies need. It's not really a dilation. It's more of a calibration, but I think the stimulation of passing the Hagar has value to help the baby more successfully, more successfully empty. Of course, in an ARM patient, I'll do dilations at 2 weeks. However, that's a colon to skin connection, whereas Hirschprung's is a colon to colon anastomosis and theoretically should have less likelihood to stricture. But I do like to pass something through just to stimulate, um, but I don't do that until it's been, uh, until it's been one month. I don't routinely put the patients on Flagyl. We have talked about, and some centers do this to preemptively give Botox, which I think is a nice idea. I haven't really started to do that routinely. We've talked about doing a randomized controlled trial on that to keep the baby from squeezing their sphincter without them knowing it too hard, leading to enterocolitis. So, so tell me again, the, the postoperative orders that you do. So do you, do you put them on, you put them on Flagyl, you put them on antibiotics immediately? I only use Flagyl to, I only use Flagyl to treat enterocolitis. I'll probably give them a pre-op dose of a second generation cephalosporin. And then maybe give them 2 to 2 post op doses and that's, that's all I do. Got it. So, uh, NPO NPO, IV fluids, um, no NG tube necessary and observed for bowel function. Mhm. And when would you start the irrigations? Oh, only if the baby demands that they need to be done, i.e., develops significant distention in the postoperative period, which luckily is quite rare. So some have described that they would actually start irrigations routinely. Have you heard of that? I, I will do irrigations routinely after an ileoanal. So in a total colonic patient, I will send them all home on irrigations for 3 months, but I don't routinely irrigate a standard Hirsch Frunk's patient unless they Force me to because they keep getting distended. I see. OK, so one unique circumstance that we haven't mentioned is the hepatic flexure transition zone, which is luckily quite rare, but I can tell you it's the most common phone call I get from a surgeon who's in an OR with a patient and is stuck, and it's they have a transition zone in the hepatic flexure, and they can't get the bowel to reach. I can tell you first of all, in that circumstance. I'm no longer laparoscoping that patient. I've opened that patient because I believe that you need to then be able to de-rotate the colon, and I don't want to do that laparoscopically because the vessels are so key and what you need to do, I don't feel comfortable doing that laparoscopically. So you need to take down the entire right colon. You need to recognize the ileocolic vessel and how it feeds onto the vessel that parallels the right colon, and very often you need to take the right colic. And of course you may very well have already taken the middle colic, and now you have an ileocolic artery with the vessel paralleling the right colon. And then if you flip the colon so that the cecum is now at the hepatic liver bed. Now you can do your pull through down the right side of the abdomen. Occasionally it goes down nicely to the left side of the abdomen, but the point is you have de-rotated the colon. If you bring it down the left side of the abdomen, you have to be very careful to have mobilized the ligament of trites so that mesenteric vessel is not draped across the third portion of the duodenum that can cause a duodenal obstruction. So you have to be able to move the colon either down the right side or down the left side. It's a unique circumstance that you face sometimes with a hepatic flexure transition zone. That's great. That's a good, uh, tip. Well, uh, Doctor Levitt, I appreciate you taking the time to go over this. This has been extremely helpful for me and I'm sure for all the listeners. We have so much more to talk about, uh, regarding Hirschsprung's disease that I don't think we can do all this in one podcast. So you and I will reconvene and finish some of the discussions specifically on some of the more complicated situations and talking about, uh, redos and things like that. So Mark, I appreciate you taking the time and we'll talk to you soon. Perfect. Thanks so much for having me. Thank you. Bye bye. We hope you enjoyed this episode of Stay Current in Pediatric Surgery. Download the Global Cat MD podcast app to receive notifications when new podcasts are released and to send comments or questions to other listeners or faculty. Also, subscribe to the Global Cat MD podcast. We'll see you next time.