The Colorectal Quiz Episode 4: Classic Hirschsprung disease - Surgical Technique

Hey there listeners, we got a brand new version of the Stay Current app, brand new layout, new content, new features. But one of our favorite new features is something that we call channels. So think of your favorite streaming service. You know how they organize content by genre? Well, we're doing that with pediatric surgery. So if you love this podcast, you can go to our colorectal channel. Scroll through podcasts and videos that have to do with colorectal surgery, or maybe you love I-PEG's content. They got a channel too. So download the Stay Current in Pediatric Surgery app. It's in the Apple App Store. It's in the Google Play Store. But until then, enjoy the episode. Last week on the Colorectal Quiz. So the next case is a one day old and presents with significant abdominal distention and bilious emesis. So I think we've discussed our basic work up and resuscitation. And now we get a plain abdominal x-ray and this is what we see. This definitely it's not your standard Hirschsprung's picture. Um so a head scratcher for Todd. Do you think it was for Dr. Levitt too? A little bit more complicated than the first case. Yeah, it's not so obvious. All right, so to finish this case, rectal biopsy, suction rectal biopsy confirms the diagnosis of Hirschsprung's disease. But I think this leads us to the next conversation. These two patients probably will have different operative approaches. Two different operative approaches for the same disease, because one is more proximal and more complicated, and the other is more distal. So be honest, did we leave you on the edge of your seat for a whole week? Well, worry no further because we're going to get into that this week. We're going to talk about the operative technique for Hirschsprung's disease. I'm Rod Gerardo from Cincinnati Children's and this is the Colorectal Quiz. Rod, you are to colorectal surgery as Michael Barbaro is to the New York Times and the Daily. So thank you for all your efforts. That's flattering. I appreciate it. Thank you. That's Dr. Mark Levitt at Children's National Hospital in Washington D.C., really just pouring on the compliments, but I appreciate it because I'm trying to do what the Daily does. Tell a story. I'm just trying to do it with colorectal surgery. Now that we all know each other, you know, Jason, you know, remember Aaron Garrison could barely tie his shoes. Here we go. Dr. Levitt and Dr. Frischer at Cincinnati Children's are talking about their former fellow, Dr. Aaron Garrison, who was also on the call. We thought it might turn into a three-year fellowship, but he uh, he was able to skive out in two. Once a fellow always a fellow. That's the truth especially with you guys. And we were lucky enough to be joined by another expert in pediatric colorectal surgery. Andrew Bateo, my partner in crime in colorectal surgery here in Washington D.C. So for those of you who haven't been following along every week, Dr. Levitt and Dr. Frischer gave us two different Hirschsprung patients. One was the more straightforward transition zone in the rectosigmoid, and then the other was a little bit more complicated, a baby with bilious emesis, and then the workup revealed a proximal transition zone. Yeah, so we really want to focus this week on the um, surgery. If you're listening to this on the Stay Current app, scroll down a little bit under the media player, you can look at the X-ray and the contrast enema for the patient that Dr. Levitt and Dr. Frischer are talking about. You know, Mark, I think the two of us talking, have you ever seen the Muppet Show and there's two old guys in the balcony? Dr. Bateo and Dr. Garrison agreed that those two Muppets were basically what Dr. Levitt and Dr. Frischer are like, so I got another bonus picture there for you. Aaron, kick us off. The baby's well, irrigations are going well, the baby's in the nicu, um smiling at you, belly's flat, biopsy showed Hirschsprung's. We discussed, should we go to the OR? Yeah, I think definitely ready for the OR. Um, you know, a patient who's in our nicu, we wouldn't necessarily bowel prep, we would probably, especially if they're um breastfed. So we would keep irrigations going though until we've got OR time. We'd have some antibiotics and some blood available just for to be safe, although typically, obviously, don't need that for this operation. And then we'd go to the OR and uh decide about our approach. Uh, with this contrast study, I think my favorite approach is to combine through laparoscopy and and transanally. So I would do a full body prep and start the procedure uh from the abdomen. And Dr. Bateo agreed. No, I mean I I like Aaron's approach. Uh, I like laparoscopy. I don't like to have surprises and if there's uh, if it's not a rectosigmoid transition, then you have the ability to biopsy elsewhere pretty easily. But what's the most important reason to consider a laparoscopic? Probably the most important thing I think though is a good deep uh laparoscopic dissection into the pelvis, so that you have very minimal transanal work to do. I think unfortunately, you know, Jason and I and you and uh Aaron and Andrea see a lot of the morbidity associated with Hirschsprung's disease and the overstretching of the sphincters, which can be done if you do an extensive transanal amount of work is really a morbidity that that needs to be avoided and laparoscopy helps with that. And Dr. Frischer is even more strict about the stretching. Previously, used to do this transanally, it would take a few hours. Now the transanal dissection should be usually way under an hour, especially in a primary pull through. Point, I just for completion sake, there are surgeons out there, we're talking to the whole world here, that don't have laparoscopy available to them. Jack Langer has showed us a very nice uh sort of umbilical approach. You can do an incredible amount of work through the umbilical approach, so I want to keep that option in mind. Dr. Garrison tried to keep us on track. Can we talk basics for a second though about like actually obtaining the biopsy, because I think there's some personally, I like using an anchor port, which I hate to give more credit to Todd, but he introduced to me. The anchor port gives you like um it's got a low profile, so there's not a lot of um instrumentation in the abdomen. It also has about an eight millimeter facial incision, so it gives you just a little bit more room to work and do the biopsy kind of at the umbilicus. So important to do a full thickness biopsy and I like to cut a square, like a cube. And if we're looking at the sides of that cube, you want to make sure that the seromuscular side is the same as the mucosal side. And then, of course, you can suture it up extracorporeally. Make sure that it's labeled appropriately for pathology because as you know, that has implications on where you make your pull through. Andrea, do you want to give us some insight how you set up if you're doing this laparoscopically, how you set up your ports? So obviously um umbilical port and then um right lower quadrant and upper quadrant ports on the right side and then switch the camera off into that right upper quadrant port and then use the uh umbilical port and the right lower quadrant as your working ports. And if you need another port because the sigmoid's very floppy, where do you put that? Um that I kind of depends right over the sigmoid really, but that that one's variable. I I definitely like using that extra little stab incision assistant port on the left side. You can make the dissection for the fellows a little easier, you can expose a little bit better. So I agree with Jason. It's fascinating. It's fascinating that you would bring that up because when you were the fellow, boy, did we have to fall over ourselves to try to make you look good. I know. I I did the memories of all of that. Okay you guys, let's get back to the matter at hand. So we have a recto sigmoid transition zone. We took our full thickness biopsy through our laparoscopic approach. Now what? Do you take the mesentery? How do you take the mesentery and how far or what do you go? I still would wait for your frozen section before I start taking mesentery. And so that I mean mobilizing the splenic flexure, making sure that what you uh are hoping comes back is uh a good news on the frozen section will reach. But I usually don't start taking mesentery until I I know for sure. So we're mobilizing, we're making sure we don't have too much tension when we finally bring our two pieces together and we're waiting for pathology. What are the absolute things that we need to hear from our pathologist so that we're good to go? Absolutely. So ganglion cells but then nerves that are less than 40 microns. So we we don't want to pull through a segment with hypertrophied nerves. Yes, and I would also add, they must see submucosa. And if you send them a biopsy without submucosa, our pathologist will say, hey, where is the submucosa? Because you want to make sure again that you don't find ganglion cells in the ceromuscular layer and hypertrophic nerves in the submucosal layer. All right, so let's say the biopsy comes back and just like Dr. Bateo says, we're good to go, then what? All right Aaron, now what is what are your moves? You said you took down the splenic flexure if you need to. You took the retroperitoneal attachments of the left colon. What are you doing actually for the mesent? Um well at that point then, you know, we're proceeding with our pull through and there's a variety of options. We've been using the Just Wright sealer a little more to dissect and take some of those um mesenteric vessels. The hook works nicely as well. We try staying just off the off the bowel, so not really getting down into the deep portion of the mesentery but as close to the bowel as you can without getting into it. And it tends to be um a little less bloody in that in that plane. And so, um, you know, we would just start somewhere where where it's easy and make a make a hole in the mesentery, dissecting through it and then cauterizing up and down all the way into the pelvis in both both leaves of mesentery. You know, I want to I want to emphasize something. Aaron, you're a very technically elegant surgeon. I've always thought that. You're making a very important point. You need to be right on the bowel because in the old days, the old swenson done through the abdomen patients were uh incontinent, non infrequently, and they had urinary retention, non-frequently. And I believe that it was related to a too wide of a dissection of the distal rectum and an injury to the Nervi Erigentes. So when you're doing this dissection, what should you consider when it comes to the arterial supply? How do you decide which vessels you need to take? You need to be very careful of preserving the arcade along the left colon and sigmoid colon so you can get enough distance to reach the pelvis. Dr. Frischer then explains that if it's distal disease, like what we're talking about here, you could just be taking distal branches of the IMA, but when you're dealing more with your left colon, you might have to take the IMA to get the left colon or splenic flexure to reach. All right, so let's move on to the transanal part of the procedure. Scroll down, click on the next image. You're going to see the transanal exposure with the Lone star retractors. And here's Dr. Bateo. Should I I think of this part is do do no harm to the dentate line, nor to the sphincters. I like to identify the the dentate line and then make a a mark about a centimeter above that into the anal canal and that's going to be the the line that we're going to divide along to start the dissection. And the pins will start at the skin. These three positions you were talking about, so you can help identify that dentate line. Then they're going to advance to cover the dentate line. And then the third maneuver is at your um where you open the mucosa and start your dissection. They'll bring that all underneath the pins and then you'll continue your dissection um superiorly. That that third move is extremely helpful. And now we talk about the pull through. And we've got options. The Suave technique or the Swenson technique, or what I see a lot of people doing what I would call to quote Dan Van Allman, the Swabson technique. Do you do a full thickness dissection or do a submucosal dissection? My preference is a Swenson, full thickness looking for the areolar plane. It's basically a bloodless plane. I don't like the submucosal dissection because I don't want to leave a cuff. Um but the Suaveist out there have been making that cuff shorter and shorter and shorter. Jack Langer will do about a one centimeter cuff now and Louise told me that he's now doing a Swenson. Um so if you're going to do a cuff because you feel compelled to do it, make it very, very short and that's something we might call a a Swabson. And you split the cuff. Yeah, you must split it. You must split the cuff. Again, Dr. Garrison was quick to point out that we might have forgotten something. Yeah, I don't think we uh clarified whether you turn the baby prone again or whether you do the operation Supine. Always great point. Personally in this kind of standard sort of patient, I I like keeping the patient supine, having the legs wrapped with the full body prep and and fastening the legs with the uh clamp to the what's that bar? The anesthesia bar. Ether screen. The ether screen, right. And so, you know, I put a little sterile bump under them and do it that way and that way there's less flipping, although, you know, for more complex cases being able to do it prone, I think is advantageous. And it it gives an opportunity for everybody get involved. The anesthesiologist can hold the clamp. Oh, sometimes you got to wake them up. All right, let's get back. So now that Aaron brought us and properly position the patient, even though we are, and in our supine patient, In our supine patient, you're in the Swenson plane, and if you've done a lot of really good work uh laparoscopically, then that dissection shouldn't take you too long and you're going to meet your dissection from above and maybe get a gusher fluid or something and you know that you've gotten into that peritoneal space. So you're you're bringing that bowel through, you'll find your biopsy site, like to go above that. How far above do you like to go? Uh no no magic number. I think you can really see that the bowel looks good. Um and that's probably influenced where you did your biopsy to begin with and then to give yourself a 5 centimeter margin or so above that is very safe, I think. Okay, so we did our pull through. We have a safe landing zone. Let's talk about the anastomosis. Um I do like putting two kind of tacking sutures on the on the serosa to the side wall. So I don't think it technically counts as like a two-layer anastomosis, but I think it just kind of anchors it in place laterally through the pelvic sidewall in the kind of three and six o'clock positions. Open up the anterior half of the anastomosis and anchoring sutures there and I I usually use 4-0 micro pops for these set up kind of 12, three and six o'clock and put the the in betweeners before transcting the the rest of the uh bowel. All four of the surgeons on the call agreed that the reinforcement layer that Dr. Garrison talked about is critical to lining the two pieces of bowel up so that you have mucosa edge to mucosa edge, which is really the key part of it. We ran out of time, but even though they were joking throughout the whole podcast, they still had time for one more. So guys, there was um there were these two kids in the holding area about to go into the surgery and they recognized each other. They were from the same class. They were both 11 years old. And the one kid said to the other, hey, um what what are you? Are you having surgery? What are you having surgery for? And he said, well, they tell me it's my appendix, I have appendicitis. And the kid said, oh, don't worry about that. I had that and I bet you're going to be going home tomorrow. You're you're fine and I'll see you back in school. What about you? What are you having done? The kid said, I'm having a circumcision. And the kid who was having the appendicitis said, oh my god, are you serious? I had that when I was a baby and I could not walk for a year. That's a good one. Mark, much improvement. Nice work. Good delivery too. Good delivery. There you have it. The surgical technique from four expert pediatric colorectal surgeons. But that was the straightforward case. Next week, we're going to talk you through the more complicated case that we presented a couple weeks ago. And while you're waiting, go ahead and check out the colorectal channel on the Stay Current app. Or maybe you've seen all that. Follow us on social media. We're on Instagram, Facebook, Twitter. Don't take my word for it. Here's Dr. Garrison. I'm just hoping to make it into one of Rod's TikToks one day. That's when I'll know I've arrived. Don't worry, Dr. Garrison. I'll get you on Tik Tok soon enough. But until next time, I'm Rod from Cincinnati Children's and remember, knowledge should be free.