Pediatric colorectal surgery. It just sounds really complex, right? These patients can come with anatomic variances, really rare anomalies, their care can be complex, dynamic, multidisciplinary, and pose a lot of clinical challenges to your everyday pediatric surgeon. I mean, just me mentioning this, I bet there's a case that popped up in your mind that was a real head scratcher that you've never seen before. At those times, it might be nice to just step inside the mind of a pediatric colorectal surgeon who's been around the block. Oh, this is really exciting, don't you think? Careful what you wish for. I mean, we've known each other for 20 plus years, and now we're doing the Zoom thing. That's Doctor Jason Fisher. He's a pediatric colorectal surgeon at Cincinnati Children's, and he's talking to one of his best friends, pediatric colorectal surgeon out in DC at Children's National Hospital, Doctor Mark Levitt. Jason was visiting Mount Sinai to check out the program, and I was giving my chief resident grand rounds on, guess what, colorectal surgery. It this is honest to true story. Their friendship, I can only describe as those two funny uncles at the barbecue. They're smiling, laughing with each other, but they're also two renowned colorectal surgeons who are clearly passionate about this specialty. So, without further ado, this is the colorectal quiz. This is a case that was sent to me by um a surgeon, um, and um I know what the surgeon did, but we're gonna play it out like you are in the neonatal ICU and there's a brand new baby, full term born with imperfor anus. He shares the screen and shows an image of a neonate, a male, with an obvious anorectal malformation. There's a little dimple there where the anus should be. All right, so this is a full-term baby, seems to be perfused well, uh, breathing room air, and obviously no anal opening. But I will tell you, it looks like there might be something there, but there's not. There's no meconium that has passed, and now we are at about 20 hours of life, and it still looks like that. So, Mark, could I pause you for a second? Yeah, during those 1st 20 hours. What are some of the key things that You would want to work up on this child as we are sitting here, hoping that we see some meconium come out from that little dimple in the anal area. Yeah, well, you know, of course, um, this is a baby with an anorectal malformation, so we need to make sure we check out any associated malformations. So, remember that mnemonic from general surgery residency, Val, so V vertebral abnormalities. Plain X-ray of the abdomen, tells you about the spine, makes sure there's no hemivertebrae. A is anorectal malformations, which is why we're having this podcast, and then C, cardiac abnormalities. We want to make sure we've checked for any cardiac defect, both on exam and on echo. E is esophageal atresia, so they ought to get an NG tube pass. R for renal abnormalities. Um, so they need a kidney ultrasound. And then L, limb abnormalities, but you should be able to find those mostly on physical exam. I also like to get a sense of the sacrum. I, I do like to measure the sacral ratio, but of course, Wait until the child is 3 months of age to make the true sacral ratio measurements, but it does give you a feel for how normal this pelvis has developed. I always worry about a missed presacral mass. Yeah, I think it's one of the other good reasons of viewing an AP view of the of the spine, because you can see if there's a hemisacrum. Of course, the spinal ultrasound, if your radiologists are attuned to diagnosing the interectal malformation patients, they ought to look at the presacral space with their ultrasound. Uh, luckily, it's pretty rare to find a presacral mass, but of course, if it's an anal stenosis or erectile atresia defect, then you You need to be very worried about finding a presacral mass and almost half the time you'll find one, and those patients will end up getting an MRI. This patient looks like a pretty typical uh imperforrate anus with no flow of meconium. And the interesting thing here to me is it looks like the dimple is pretty normal looking and the buttock looks pretty well formed. But despite that, we're at 20 hours with no meconium. So what's the next step? We typically get at about the 24 hour mark, give or take a few hours, a cross table lateral to see what does the gas column look like. Uh, in this patient. Is there a low gas column that looks like it's almost touching the skin, or is it pretty high up? And that might dictate what your next steps might be. And obviously, during this time, we're also making sure the patient is doing well, getting resuscitated, and that the abdomen's not getting distended to a point that we need to intervene before waiting this 24 hours for a cross table lateral. Yeah, no, I agree, and of course it's important that if this baby was born today, we're not rushing to do anything, because we need time to have this baby declare themselves to either be someone who's gonna need a colostomy or someone who might be able to benefit from a primary repair, a meconium, if a if a perineal fistula bubbles up. Then Doctor Levitt pulls up the cross table lateral film. It's important to note, and I didn't mention that the baby is now prone, putting the buttock really at the highest point where air will rise to. And you can get, you can get this X-ray at the bedside in the neonatal unit, just put the baby on a, on a bump under the buttocks. I, I think it's important and it's pretty glaring in this X-ray, that you mark where you think the anus is appropriate, so you could get your measurements and get an understanding of what the distances are going to be depending on your next steps and potential operative uh repair or approach. Yeah, I don't know what they put there. It looks like a Might be a silver dollar. Yeah, uh, that could, that could be. First of all, it's a beautiful image. It's very, uh, straight on lateral. The air column has really risen very nicely, and boy is that thing close to the perineal skin. It's right there, isn't it? It's an impressive film. It's a great one. Yeah, the other thing I would note, um, again, and I wouldn't calculate a sacral ratio at this stage, but the sacrum looks quite normal. I bet this kid is gonna have a pretty normal acal ratio, certainly greater than 0.7. Which connotes a very good prognosis for bowel control and really peace of mind for the family. They want to know what's going to happen to this baby in 4 years when they need to potty train and go to school. Just adding to the prognosis and just putting the picture together. When we looked at the baby, the image of the baby, and, and looking at his perineum at that. Point. It did look like there is a well-formed buttock, good muscle. It looked like there was a true area where you could determine where the sphincter mech mechanism was going to be located in alignment with also seeing a well-developed sacrum and all these other things. All these things lead together to likely a good prognosis. So you have a baby with a low rectum, an air column that is just about 8.8 millimeters from the skin, a normal sacrum, and buttocks that appear to be well developed. So, What's next? Well, I have two answers for you, Mark. I think if I was sitting in a hotel room, being examined by a bunch of board examiners, my answer would likely be a diverting stoma in real life, which is what we're discussing here. What would we do in, in, uh, on a Tuesday afternoon? I, I think. This air column is, if you're gonna get this X-ray and base your decision making on where the air column is, and what's going on. I'm pretty apt to say, I might perform a primary posterior sagittal inter ectoplasty with this type of imaging in front of me. You know, I think, I think the key to deciding whether to dive into a perineum and posterior sagiti is, where is the rectum? You wanna know that the what structure you will find if you make a posterior sagittal or even a mini posterior sagittal incision. And there is no question in my mind that if I open posterior sagita here, the first structure I would find would be rectum, because the danger is that you go in posterior sagita, you don't know where the rectum is, and you find something midline and white, like the urethra, the bladder neck or the bladder itself. And that's why we do colostomies, and that's why we do distal colostograms. So we know exactly where the rectum is, and we know whether we should approach it perineally or whether we should do it laparoscopically. But in this case, we know we're gonna find the rectum. Maybe we should just do a primary repair. It, it goes without saying and, and one of our, I think one of our big teaching points is you should never try to go in blind. Right, we're going in with knowing that if we make a posterior sagittal incision right now, the first thing we're gonna hit is that air pocket, which is the distal rectum. So I will tell you that the surgeon who sent me this case, they did not do that. They felt more comfortable doing a colostomy, and I wanna tell you, that was the safe thing to do. Bravo to them. That was the right choice. Nothing wrong with that at all, but of course, we are giving the child a colostomy. And whatever complications can happen from that, and we are giving the child a colostomy closure, and whatever complications can happen from that. So everything in medicine is a balance, but there is no question that the anal part has been made safer by having a colostomy. The next image blows Doctor Fisher's mind. Doctor Levitt pulls up the distal colostogram, very clearly, there is a low bulbar fistula. So, this is sort of scary, because if you went in and grabbed this rectum and did a primary anoplasty. And did not know there was a fistula, and unfortunately, I have seen this done by some very good surgeons where they went in, did a beautiful anoplasty, but ignored the fistula, and the child down the road started peeing out their anus. And that's what question mark. We both agree that probably in real life, we would have done a primary posterior sagittal approach on this patient, and we would have found the rectum, and we could have made that rectum reach the perineum and done our anoplasty. How do we handle this? Because I didn't know that there was a fistula there. This is troublesome. Yeah, I mean, I think that's, that, I think that's the key point of this case, that I think a lot of people would have done a primary repair because they said, ah, it's a chip shot, the rectum's right there. But when I do that, I open up the posterior wall of the rectum and I inspect the anterior wall of the rectum. So, for Doctor Levitt, he dissects the little bit of the anterior wall, carefully lifting it off the urinary tract. For him, in a patient like this with a low rectum, this will usually rule out the fistula. I think two other points I want to put is that you can see this fistula is very close to the rectum. And if you're going to dissect that free, and along the urethra, um, it's important to make sure that you know that those two structures are not very far apart, and getting that into that proper plane is very important. I'd also like to mention, um, the fact that we said this is a bulbar fistula. The reason why Doctor Levitt or Mark is saying that. It's a bulbar fistula, it's at the elbow. You can see it's at the elbow of the urethra. And tell us that the uh the nomenclature we like to use is an anatomic pure anatomic nomenclature saying that it's a bulbar fistula. My final question for you, Mark, or, or point is, what if this patient had trisomy 21? Would you have had a different thought process or approach? It's funny you say that question today. I got an email today from a surgeon who said I have a he has a baby with Down's and imperforate anus. Does he, does the baby need a distal colostogram, or can he assume that the baby has no fistula? And I said, no, the baby might have a fistula. 95% of Down's patients have no fistula, but 5% do. So I would still do a distal colostogram, and some of them might have a fistula. Alright, let's summarize the case. So first, make sure that you do a complete workup. There are a lot of associated anomalies with that. Make sure you also get a preoperative cross table lateral X-ray to evaluate the air column and help you with surgical planning. Once you start to look at everything, keep in mind that even if it looks like a chip shot PARP, colostomy is still a reasonable and safe choice in the meantime. Now if you do decide to go with the PAP, make sure to rule out a fistula intraoperatively. Now, here's some closing thoughts from Doctor Fisher and Doctor Levitt. Hey, I got a joke for you. Oh, go ahead. Have you seen the movie about constipation? I have not. It hasn't come out yet. Oh man, OK, thanks my friend. It was. I hope that you guys enjoyed the first of potentially several colorectal episodes. If you're listening to this on your podcast app, great. Thank you for listening, but You're missing out. So download the Stay Current Pediatric Surgery app. You can listen to this podcast and click to see the images that Doctor Fisher and Doctor Levitt are talking about. Then you could click over and watch some technique videos, or watch a video about colorectal surgery, listen to other colorectal podcasts. It's all there in the Stay Current app. Until next time, I'm Rod Gerardo from Cincinnati Children's, and remember, knowledge should be free.
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