Hirschsprung Part 1 podcast.mp3

Pediatric Surgery

Dr. Ponsky: 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 Hirschsprung’s Disease and with us today we have definitely an expert in the field, Dr. Marc Levitt who’s at Nationwide Children’s Hospital. Marc, thank you for joining us today.

Dr. Levitt: Hi Todd, it’s great to be here.

Dr. Ponsky: Marc, before we get started, can you give everyone a quick synopsis of where you are and what goes on down there at Nationwide?

Dr. Levitt: Sure, I’m at Nationwide Children’s Hospital in Columbus, Ohio, United States, the very center of the state of Ohio, in the Midwest. I’m a pediatric surgeon; I’m the surgical director of our center, which is a center focused on the collaboration between pediatric surgery, pediatric urology, pediatric gynecology and GI motility, or all problems that could possibly relate to the colon and pelvis. 

Dr. Ponsky: Perfect. How long have you been there in Columbus?

Dr. Levitt: We’re about to celebrate our first year anniversary.

Evaluation of neonatal distal obstruction (6:57)


Dr. Ponsky: Perfect, and I know that 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, Marc, we’re going to have some fun here and we’re going to go into the first patient. So let’s start off with a newborn who has Hirschsprung’s Disease. Marc, 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 patient and before you start asking questions, just as you walk in, you notice that the patient has pretty impressively distended abdomen. How do you evaluate these patients with this type of evidence of a possible distal obstruction and what’s your usual method for working this up.

Dr. Levitt: 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 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 bilious 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.


Rectal irrigation (08:17)

Dr. Ponsky: So when you take the baby you get your contrast enema and there is suspicion for Hirschsprung’s Disease. There is what looks to be a transition zone around the rectosigmoid junction. What is your next step with this child?

Dr. Levitt: 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 probably the best treatment for Hirschsprung’s disease is irrigations. It is very rare that Hirschsprung’s disease is a surgical emergency but if you don’t irrigate and overcome the distal obstruction, it will become an emergency. And I can’t emphasize enough how important it is to know how to do a proper irrigation.

Dr. Ponsky: Can you go through that exactly how we should be doing these irrigations? 

Dr. Levitt: 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-Luer lock syringe and I basically insert about 10-20 cc aliquots at a time, passing it with a lubricant of course, instilling saline, moving the tube to and fro, advancing it as far as you can without meeting 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 that you’re getting meconium out, you’re getting the tepid, 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 for several days.

Dr. Ponsky: So Marc, just to clarify, you take your 60 cc syringe through that 12 French tube, and by the way I use actually.

Dr. Levitt: I would use a 20 French tube.

Dr. Ponsky: 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, large catheter, that’s probably one of the biggest mistakes I think I’ve seen, is when I end up showing up to see what they’ve been using and it’s too small of a caliber tube. You then said that you inject 10-20 at a time, do you inject 10-20 at a time, let that drain out, and then put in the next 10-20?

Dr. Levitt: Yes.

Dr. Ponsky: Okay.

Dr. Levitt: 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 enema.

Dr. Ponsky: That’s right.

Dr. Levitt: Babies with Hirschsprung Disease have no ability to expel the enema fluid and they just will walk away after 100 cc have been instilled and you haven’t accomplished anything.

Dr. Ponsky: So do you find yourself manipulating the tube, sliding it in and out slightly, massaging on the abdomen, or does that not work?

Dr. Levitt: Yes I absolutely would do that. Keep moving the tube into various positions. 

Dr. Ponsky: Marc you have this baby and you’ve been doing these irrigations. Let’s say for whatever reason you don’t want to operate on them right now. Let’s say they’re really small, they’re premature, or whatever reason. Do you ever send them home with irrigations or do you keep them in the hospital? 

Dr. Levitt: I am not a fan on sending babies home on irrigations because it’s not as reliable as doing the irrigations in the hospital and then operating. If you have a particularly responsible family, that is not an unreasonable option.

Dr. Ponsky: Okay, perfect. Marc, what if you don’t have a pediatric pathologist at your hospital? Can you just do the pull-through based on the contrast enema?

Dr. Levitt: Yeah, 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 are somewhere in the world without pediatric pathology, and you need to intervene on a baby that’s sick, not responding to irrigations, and you do not have pathology, then you need to divert that baby. My personal recommendation is to divert that baby in the ileum.

Dr. Ponsky: So tell me how you do your biopsies.

Dr. Levitt: I take one sample. As long as you’re in deep enough, it’s important to remember that you need to be at least 1 cm in from the dentate line. If you biopsy too close to the dentate line, everyone has an aganglionic segment there, and you could get the wrong answer and someone thinks that the baby has Hirschsprung’s Disease when they actually don’t. You have to be careful not to biopsy too high. If the gun gets sent in too deep and biopsies at 4 or 5 centimeters, which I have seen, happen, you may miss a lower zone of Hirschsprung’s Disease.

Dr. Ponsky: So can you go through and explain what you want to see on these biopsy results? 

Dr. Levitt: The pathologist really needs to be prepared for evaluation of Hirschsprung’s Disease. A U.S. surgeon cannot accept a pathologist report unless there is the absence of ganglion cells and the presence of hypertrophic nerves. The absence of ganglion cells alone is not Hirschsprung’s disease: that could be a biopsy that’s taken too low.

Dr. Ponsky: What about using acetylcholinesterase?

Dr. Levitt: That is a reasonable adjunct. It’s not absolutely vital, and it’s not done at all centers. A lot of pathologists like it because it’s confirmatory. But, the standard of care that you must have is a traditional H&E whether there are ganglion cells or not and whether the nerves are hypertrophic. That’s usually enough, and that’s pretty much what we do here.


What if pathology is not available? (14:14)


Dr. Ponsky: Okay so this is the same baby now. The irrigations are not working, the distension persists, what do you do at this point? 

Dr. Levitt: You cannot have that baby remain distended because distension is stasis and stasis is the problem with Hirschsprung’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 Hirschsprung disease has an immune component to it, and 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 that stasis in a Hirschsprung’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 can 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 Hirschsprung’s, usually left colon or transverse colon, or right colon, or total colonic Hirschsprung’s disease. If irrigations are not going well, and the baby is ill, that’s luckily very rare and you need to do something. That’s when the discussion of whether to do a stoma comes up, and then there’s discussion about going into the operating room and bringing out the dilated segment which is a commonly done move in the developing world which is a very reasonable and a very safe thing to do even without pathology because 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 3:00 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 past 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 and an ileostomy and wait for permanent section which is much more accurate and then at some point down the road do your pull-through.


Different surgical techniques (17:17)


Dr. Ponsky: When do you time the operation and how do you prep them? 

Dr. Levitt: I find the soonest elective time to schedule that case in the next several days. I don’t do any GoLYTELY prep or anything like that, I just make sure they’re irrigated well from below.

Dr. Ponsky: Okay. Now let’s talk about the different operations. We have Swenson and Soave and Duhamel. Can you go over your thoughts on these different surgeries? Let’s talk about Swenson.

Dr. Levitt: 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 full thickness down below the peritoneal reflection and then pulled-through colon. It was a beautiful operation. I believe it 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. This was something 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. To respond to that, 2 very brilliant ideas came up: one was from Dr. Soave in Italy and one was from Dr. Duhamel from France. What Suave did was through the abdomen, and I think it’s important to recognize that these original operations were done transabdominally; the dissection 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, Soave came in a little bit tighter, and essentially did a mucosal dissection. That was quite brilliant and that sort of became the standard and the original Suave, actually, once the pull-through was done, Dr. Suave would actually pull through the colon and leave it coming through the kid’s anus for a week and would go back to the operating room and do the colo-anal anastomosis. It was actually Dr. Scott Bole who said 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 perineal reflection, and the ganglionic portion gets pulled in a retrorectal position, and then connected so the 2 lumens are mated into 1 lumen by a stapler, also a transabdominal operation. Those became the 3 operations and Swenson really took a back seat because people thought it was fraught with complications. I would also add American surgeons don’t know a lot about the Rehbein 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 bowel made it to the rectum with approximately a 4-6 cm original rectum sitting there that’s aganglionic. Fascinating actually that of those 4 procedures, the only one that leaves behind virtually no Hirschsprung’s is the Swenson. The Soave leaves behind the outer rectal wall, the Duhamel leaves behind the original rectum, and the Rehbein leaves behind the original rectum as well. Amazingly, many of those patients did amazingly 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 Hirschsprung’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 primary operation essentially with no initial colostomy. And I would give Henry So 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. Out of desperation he just went ahead and did the entire operation I just described primarily, and it worked. The next aspect of history, and I think it’s important, I’m skipping through decades of history, this is very well described actually there’s a very nice article written by Dr. Grosfeld on the history of Hirschsprung’s disease which I would strongly recommend people read, 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. Keith Georgeson is the one who said, "I have laparoscopic skills, let’s apply it to Hirschsprung’s disease.” What he did is he delineated the colon and did all the colonic work elegantly, laparoscopically, with a Soave dissection from above. The concept then became this transanal approach. The transanal approach, which everyone now talks about, was an absolutely brilliant idea that Luis De La Torre and Jack Langer came up with during the same period of time. It was starting the dissection from below, intending to do a primary operation. I will tell you, this is history that no one really knows and probably doesn’t need anyone to know, but in my time working with Dr. Peña, we were really into the full thickness rectal dissection because that was the same dissection one used for a standard PSARP, which Dr. Pena obviously invented and we said well why don’t we do the same for Hirschsprung’s disease. I still remember that we did a couple posterior sagittal incisions to mobilize the rectum through a posterior sagittal incision full thickness. We were very proud of ourselves until the De La Torre and Langer idea came out and we said why did we make a posterior sagittal incision, because you can do everything that you need to do transanal. So the transanal now, and we of course 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 the planes, and that’s probably the most common operation that’s done.


What does Marc Levitt do? (25:25)


Dr. Ponsky: Okay so that was a great review of all the different technical options available for a Hirschsprung’s pull-through, but Marc, what do you do in your practice?

Dr. Levitt: More recently, I have started to do about 10 years ago, a transanal Swenson. So when you say Swenson you have to remember that was transabdominal, when you say Soave that was transabdominal, so I think it’s appropriate to say transanal Soave-like or Soave-plane or transanal Swenson-like or Swenson-plane. 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 Hirschsprung’s except for the 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 one has known, and that we talked about before. Dr. Swenson himself, who recently died at the age of 105 I believe, was a big fan of helping to remind us 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. He was very thankful that we were repromoting the Swenson and a number of centers have now taken that on and enjoyed doing the Swenson. I think it’s a cleaner, neater operation. The Soave is absolutely beautiful of course, and what you’ll notice actually is people are making the Soave more Swenson-like over time. Even the Soave enthusiasts are making shorter and shorter cuffs. Dr. Georgeson’s original description of the laparoscopic Soave, which essentially was the laparoscopic pull-though, recommended a 5-cm cuff. Nowadays, Langer and De La Torre would do a 1-cm, maybe 1.5 cm cuff, essentially a Swenson. So one could call it perhaps a Soaveson. 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.

Dr. Ponsky: Marc, that was a great overview of all the different techniques. Is there a place that you can think of off the top of your head where people might be able to go and watch demonstrations of these techniques, or any videos yet available online or for purchase?

Dr. Levitt: I’m happy to share my video of how to do the transanal Swenson-type dissection. Anyone is welcome to email me and I will send it to them. It’s marc.levitt@nationwidechildrens.org anytime.


Laparoscopic vs. transanal approach (28:24)


Dr. Ponsky: Let’s talk about the laparoscopic approach, the approach that I prefer. What are your thoughts on that? I know there is a lot of controversy.

Dr. Levitt: Laparoscopy is a fantastic adjunct to Hirschsprung disease. I think the controversy really becomes do you apply laparoscopy to all Hirschsprung’s patients, or are there some where it’s more appropriate to do transanal only. My feeling about this has definitely changed because I’ve seen a lot of morbidity that has resulted from an overly aggressive transanal-only approach in trying to reach the transition zone with the valiant attempt at never going into the abdomen laparoscopically or by laparotomy, and I think that is a problem, to be honest. In my view, if you have a very reachable transition zone, comfortably, sort of mid-sigmoid, and by the way in many babies it’s not obvious, but if it’s very obvious which happens from time to time, and it’s mid-sigmoid, 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. 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 PSARPs, 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 am very confident in the location of the transition zone and I can reach it transanally. Anything else, I will do laparoscopy first and make sure I know where the level of transition is. For those out there that do Duhamel procedures, 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 were a total colonic Hirschsprung’s situation. Those patients are at risk for having burned your desired bridge leaving the rectum in position. I personally do ilio-anal anastomoses in total colonic Hirschsprung’s, so for me, it doesn’t burn a bridge. However, for those who want to do Duhamel procedures for ilial Hirschsprung’s, then they don’t want to do a transanal at all, although I will tell you that you should pretty much be able to figure out if you’re dealing with a total colonic patient. If you have a very typical Hirschsprung’s presentation, a pretty obvious looking transition zone, a patient who has responded well to irrigations, that’s not going to be a total colonic case. Total colonic patients 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 location. So I think that’s the controversy. 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, 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.

Dr. Ponsky: You know, Marc, the GlobalCastMD 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 they should all be done laparoscopically. I am not convinced that laparoscopy is more invasive than pure transanal. It’s just so easy and elegant to get the entire dissection done without pulling on the rectum, so I do them all that way.

Dr. Levitt: I would agree with you, and I’m particularly persuaded by the fact that if you do a transanal and you’re too aggressive in your attempt to visualize the dissection, you would have been much better off with the lower laparoscopic dissection, no question about it. Having said that, if it’s reachable, and I feel that I could 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 and not in too deep, you’re really doing a beautiful operation. But in 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. 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 marginal arcade that goes down the left colon and sigmoid, and 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.


Technical pearls (33:44)


Dr. Ponsky: So let’s talk about some tips and tricks for preserving the continence mechanism. What do people do wrong? What are some of the pitfalls that you can advise against here?

Dr. Levitt: 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.

Dr. Ponsky: That’s a great pearl, Marc. So up until now you’ve given us a great review of the history of Hirschsprung’s Disease, the work-up 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?

Dr. Levitt: I like to use the Lone Star retractor, which are those pins that give you a circumferential display of the anal canal. 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 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, and then we put the stitches circumferential. I put 5-0 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 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 you don’t want to hurt the sphincter.


Is it safe to leave 1 cm of Hirschsprung bowel? (36:06)


Dr. Ponsky: Marc, 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 cm deep to the dentate line. Is it a problem leaving that 1 cm of Hirschsprung’s aganglionic bowel?

Dr. Levitt: By definition you’re leaving behind 1 cm of columnar epithelium, that of course is Hirschsprung’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. The purest operation is then to start that dissection Swenson, but you can start that dissection right then and there as a Soave, but either way you must preserve the anal canal and the dentate line. Of course, if 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 retro-rectal position.

The dissection (37:25)

Dr. Ponsky: Now you’ve marked out 1 cm proximal to the dentate line, now how do you start your dissection?

Dr. Levitt: Once I have that exposure, I’ve marked my purple line, I’ve put 5-0 silk circumferentially, 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 full thickness; there’s a nice areolar plane that you find, and once you get good at it, you will love that plane. 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; it’s a very similar dissection to the PSARP technique when you mobilize a rectum. You want to get inside that whitish fascia, and as you continue to mobilize, the rectum frees up. Remember, the rectum doesn’t really have a mesentery. The rectum’s blood supply is intermural, so as you get higher and you reach the peritoneal reflection, 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 prone position, is I recognize that the anterior rectum frees up much quicker than the posterior rectum, and I take advantage of that fact. I will break into the peritoneal reflection, 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 reflection anteriorly, often you can pull the sigmoid out. You can actually do a biopsy of that sigmoid full-thickness, and then shove it back in, and while the pathologist is spending their time analyzing that specimen over the next 40-45 minutes, you continue your posterior rectal dissection. If you time it well, right when you’re done with freeing up the entire rectum the pathologist calls and says you have good location and 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 like that 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.


Laparoscopic biopsy technique (40:05)

Dr. Ponsky: Marc for those who prefer to do the laparoscopic approach, can you describe how you do your laparoscopic biopsies? 

Dr. Levitt: 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 anorectal malformation or Hirschsprung disease. Then, in the umbilicus you can put a grabber and grab various parts of the colon, pull them out the umbilicus, and then do a full-thickness biopsy and drop it back in. What a lot of people do is they do a laparoscopic biopsy so they grab the seromuscular 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 ganglion cells, when the submucosal layer that 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.

Dr. Ponsky: That’s interesting. I’ll do my laparoscopic biopsy the way you described and I send my specimen as 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 Marc.

Dr. Levitt: I would definitely, 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, and it saves a lot of time. If you do it laparoscopically, if you do it through the umbilicus it’s very easy to do a full-thickness and then sew it up, but that is only going to tell you ganglion cells in the seromuscular layer; it’s not going to tell you whether your submucosa is okay.

Dr. Ponsky: I never realized that you could have good ganglion cells within the seromuscular layer but no ganglion cells in the submucosal layer.

Dr. Levitt: You may have ganglion cells in the submucosal layer, but you might have hypertrophic nerves there. Therefore, you then you basically have set up a transition zone pull-through, and you want to avoid that. So, if you were 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.

Dr. Ponsky: I love it. Marc, so let me just describe the way that I like to do it laparoscopically because we do it different. I put in 3 ports: I put the scope in the umbilicus and then I put a 3-mm just above that, and then one on the right lower abdomen, and I do the laparoscopic biopsies with scissors. It’s actually a fun technique to learn to do and it’s a fun exercise for the trainees as well. Then I put a stitch, you don’t need to, but it’s a nice exercise in intracorporeal tying as well. 

How to make sure you are not in the transition zone (43:19)

Dr. Ponsky: Do you get a confirmatory biopsy, and I’m making a guess your answer is no, very low down before you do anything laparoscopically? Do you go down in the peritoneal reflection?

Dr. Levitt: No. If I already have a rectal biopsy then I know it’s Hirschsprung’s. I like to try to do this operation with one biopsy. If you pick the right spot, if you pick the healthy spot, in usually the proximal sigmoid or the left colon.

Dr. Ponsky: Two centimeters about where you see the transition?

Dr. Levitt: Yeah, something like that. I want to make sure that our pathologists are not just 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 like to know the microns: the microns should be 40 microns or less. Anything bigger than 40 microns is transition zone bowel, and I go a little bit higher. I think the concept of go 5 cm above is inaccurate. I think transition zone is a spectrum: I’ve seen 10 centimeter transition zones; I’ve seen 3 centimeter transition zones, so you really need a confirmatory biopsy and your pathologist can, if properly trained, get you the ganglion cell information and also the quality of nerves to the micron measurement.

Dr. Ponsky: Marc, 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?

Dr. Levitt: Yes, 40 microns or less I would consider normal.  

Dr. Ponsky: Right, anything above that you’re concerned that they’re hypertrophic?

Dr. Levitt: Yes.

Dr. Ponsky: Okay, and that also answered my other question. I know some people do 3 biopsies at a time and send them all so they don’t waste time. I agree with you, I try to do as few as possible.

Dr. Levitt: Yeah, I just do one biopsy in the good bowel. I already know it’s Hirschsprung’s. You know this is sort of bringing up the point of how high to go, right? So for transanal or laparoscopic where do you go? I mean if you could get away with just mid-rectum, is that enough? In most of these patients it’s somewhere in the sigmoid, and you bring down the proximal sigmoid to do your colo-anal anastomosis. I think 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 down into the perineum. Makes for a very easy to irrigate baby. And I recognize that that might take a little extra of the bowel that you didn’t necessarily need to take but 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 who have not had enough of a pull-through, where the entire sigmoid loop is still there and they need a re-do to remove more of that.

Dr. Ponsky: Marc, do you start the dissection before you have your biopsy results back? While they’re waiting to do the path are you doing your laparoscopic rectal dissection?

Dr. Levitt: If it’s a very obvious transition, yes I would start. If I’m worried at all about a higher zone, I would say honestly anything proximal to the splenic flexure, I personally would do colonic biopsies and ileostomy and wait. Because frozen section has notoriously been fraught with errors in those cases and there is no urgency. 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 about the transition zone, the bowel looks beautiful, and 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 or, usually, I will divert with an ileostomy so the baby can thrive and do well quickly and wait for permanent section. In a classic sigmoid situation, then you do your standard pull-through that we’ve talked about.

Dr. Ponsky: Okay. Before we get onto 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?

Dr. Levitt: Yes. One thing I wanted to mention is that if you’re going to do transanal only and you’re in prone position, when you free up the anterior rectal wall, and then we talked about how you know we go to the posterior waiting for the biopsy, what I like to do is I put sequential stitches on the inferior, 6:00 position, of course you’re in prone position, so 6:00 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 mosquitos, but also keeps the bowel aligned. It makes it less likely that you’re going to spin the bowel. 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 get to a put where the sigmoid just comes right out of the abdomen, and you have 3 or 4 stitches that help you do that, and they’re all at the 6:00 position, and it helps you from twisting the pull-through.

Dr. Ponsky: Now for me, who does this laparoscopic so my child is supine, do you still do that but then at the 12:00 position?

Dr. Levitt: Yeah. It’s a nice thing to do, and, because I’m so neurotic about that detail, at the very end, I’ll pass a tube, and make absolutely sure that I did spin it 360 degrees.

Dr. Ponsky: I like that. By the way, for the setup for the laparoscopic approach, if you’re supine, someone showed me a trick, I think this was also Scott Boulanger, of tying the feet to this mobile sort of ether screen type of thing that goes across the table, and you put the feet up or down based on if you’re going from below or from above.

Dr. Levitt: That’s a good way to do it, and you put a little foam, a bump, at the bottom. You know as far as laparoscopy, I don’t know if we completed where all the trocars go. 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.

Dr. Ponsky: I think that’s great, so you actually put in 4 ports then. Is that correct?

Dr. Levitt: Yeah. I’ll use 4 ports, like a bladder neck fistula for ARM and for Hirschsprung’s because I really like holding up on the sigmoid by 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.

Dr. Ponsky: Yeah. I do want to point out that for both the way you and I both described it, the operating surgeon is mostly on one side of the patient. So you were on the right side, your ports were sort of on the right side of the abdomen. A lot of people feel that the rectum is midline, so I’m going to put my left hand on the left side and my right hand on my right side. It’s actually quite tricky, would you agree?

Dr. Levitt: It’s very uncomfortable. 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 actually make them stand there before we prep so that they’re not pushing themselves into the anesthesia machine after everything is set.

Dr. Ponsky: I like your tricks on learning here it’s actually been helpful. The one thing you know that we, thanks to you we recently hired Dr. Aaron Garrison from when he worked with you in Cincinnati, and, I hope he doesn’t listen because I’m going to say that he’s one of the most phenomenal surgeons that I’ve worked with, he is actually convincing me to start moving from the Soave to the Swenson, so listening to you describe all of this, I’m starting to drink the Kool-Aid and become a believer.

Dr. Levitt: 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, you’ll recognize it is much more pleasant than the Soave: it’s much more elegant; there’s no bleeding at all. But I would strongly recommend trying transanal prone, because it’s a really neat way to look at the rectum. Of course, if you do most of your work first laparoscopically, I don’t think you need to go through 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 just be doing that prone.


Postoperative management (52:48)

Dr. Ponsky: Yeah. I think that’s a great tip. Marc, what do you do with these patients post-operatively? What do your post-operative 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?

Dr. Levitt: I’m very conservative about the post-operative period. I find that often patients are fed too early with Hirschsprung’s disease and many of them go home. I am passionately committed to not have a baby come back and be readmitted with enterocolitis. What I will do is wait until the belly is absolutely soft and flat, they’re having bowel function: passing gas and stooling. I will get an x-ray because I think abdominal distension is sometimes subclinical. I don’t feed that baby until the x-ray looks good and the belly is flat. And that usually takes 3 or 4 days. I know there are people who will feed those babies on days 1 or 2, and I think 9/10 times that works beautifully. But if that baby eats, a little bit distended, and goes home, they can come back with enterocolitis. I don’t want any baby to come back with enterocolitis, so a couple of extra days to me is not a big deal to wait and not take 1 step forward and 2 steps back. I make sure the families know how to do irrigations pre-op, I make sure they have their supplies and know what an irrigation is, and I make them paranoid about distension, 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 irrigation thereafter if the baby needs them, which is rare, but sometimes necessary. My routine has been at one month to check the anus in the clinic with Hagar dilators, not my finger, and then dilate. Most babies need it: it’s not really a dilation; it’s more a calibration, but I think the stimulation of passing the Hagar has value to help the baby 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 Hirschsprung’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, but I don’t do that until it’s been 1 month. I don’t routinely put the patients on Flagyl. We have talked about, and some centers do this, preemptively giving Botox, which I think is a nice idea. I haven’t started to do that routinely. We’ve talked about doing a randomized control trial on that to keep the baby from squeezing their sphincter too hard without them knowing it leading to enterocolitis.

Dr. Ponsky: Tell me again the post-operative orders that you do. Do you put them on Flagyl? Do you put them on antibiotics immediately?

Dr. Levitt: No, 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 2 post-op doses. That’s all I do.

Dr. Ponsky: Got it. So, NPO.

Dr. Levitt: NPO. IV fluids. No NG-tube necessary and observe for bowel function.

Dr. Ponsky: When would you start the irrigations?

Dr. Levitt: Only if the baby demands that they need to be done, i.e. develops significant distention in the post-operative period, which luckily is quite rare.

Dr. Ponsky: Some have described that they would actually start irrigations routinely. Have you heard of that?

Dr. Levitt: I will do irrigations routinely after an ileo-anal anastomosis. In a total colonic patient, I will send them all home on irrigations for 3 months, but I don’t routinely irrigate a standard Hirschsprung’s patient, unless they force me to because they keep getting distended.

Dr. Ponsky: I see.


Transition zone in the hepatic flexure (56:45)

Dr. Levitt: 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. 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 be able to de-rotate the colon, and I don’t want to do that laparoscopically because the vessels are so key in what you need to do. I don’t feel comfortable doing that laparoscopically. 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. Of course, you very well may have already taken the middle colic, and now you have an ileocolic artery with the vessel paralleling the right colon. 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 the left side of the abdomen nicely, 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 Treitz so that mesenteric vessel is not draped across the third portion of the duodenum that can cause a duodenal obstruction. 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.


Additional Content

Discussion with Dr. Marc Levitt and Dr. Todd Ponsky regarding evaluation and management of Hirschsprung disease.