OK, we're back. This is part two of the last intestinal rehabilitation podcast episode that we started. Uh, just to give a reminder, we were talking about the step procedure for patients with shortcut. We talked about the indications for doing it, which patients would benefit from the step procedure, and we even went through some of the operative techniques. With Dr. Wales. In this part two, we're going to talk about some postoperative considerations. As a reminder, I'm Ellen and Cisco. I'm Anton Bash, a research fellow at Cincinnati Children's Hospital Medical Center, and this is Stay Current podcasts. And in this series, we're talking to two of the surgical experts in pediatric intestinal failure and intestinal rehabilitation. We have the surgical director of the intestinal rehab program. I'm Paul Wales. I'm a pediatric surgeon here at Cincinnati Children's Hospital, and We have the former surgical director of the intestinal rehab program. I'm Michael Helmrath. I'm a pediatric surgeon also doing intestinal failure surgery and research here at Cincinnati Children. If you haven't listened it yet, make sure to listen to part one of this episode. OK, but let's jump in. There's a lot of enthusiasm about different things at different times. The step procedure would be an example of that. Like any new tool in our toolbox, everyone jumps on a bandwagon. And then over the subsequent years, you start to figure out what the real indications are. So Paul, what do you expect from a step? What do you tell families that your expectation is in their benefit to enteral nutrition from TPN and what's the timeline you talk about? So, I think we have to remember that the biggest benefit of the step, for instance, is the fact that you're tapering the bowel and reestablishing a more normal caliber to improve motility. OK, so what Dr. Wales is saying here is that when you Do the step procedure and you're tapering the bowel, you're getting it more to normal caliber. What this means is you're not creating a new bowel, but you are redistributing it in order to reestablish more normal caliber bowel, which helps improve motility overall. So the mucosa heals and ultimately and hopefully functions better. So, a lot of these kids will have dilated bowel, and that's often the impetus for referral, but if the child is adapting and they're making. Progress. I don't operate. But if they're not making progress and maybe they're having a lot of complications for being on chronic TPN, such as liver dysfunction, then maybe we should look at their anatomy, their bowel anatomy, and think about doing surgery. But Paul, the kid can't tolerate any feeds. He's on 2 cc's an hour and still throwing up. And so what Dr. Helmarth is asking is, what if we have a child who's on a really low rate of feeds but still throwing up? What do we talk about then? I tell the family, I describe the procedure. I The outcomes, it can take up to 6 months before you actually start to see a significant improvement in absorptive capacity. And how do we measure improvements in absorptive capacity of the bowel? That's measured with fecal fat, alpha-1 antitrypsin clearance, xylose absorption, and even citrulline levels as they rise over time. It doesn't not happen overnight. And I think the reason for that is that inflamed, sick, leaky mucosa that you have in the setting of bacterial overgrowth. To heal, and Dr. Wells says that overall we might even see a reduction of parental nutrition support, about 50%. Half of the people that have a step will have progression of improved animal tolerance. Half will have actually a worsening. And so that six month time Paul is talking about is very critical for you to analyze how the patient is moving forward. So Dr. Helmroth is saying here is that the decision to do a step procedure requires a lot of thought beforehand. He says that he doesn't necessarily do them initially if they never really tolerated internal feeds, for example. If you're going to operate on a kid for a step procedure, you need to first rule out other anatomical problems. And if your mind is just focused on the step and you don't lay out the bowel and get the mesentery completely oriented. and see your anatomy before you make any decisions, you will miss some of the reasons why these kids aren't getting better and just add a step on top of a problem that you're not going to solve. The question would be, Michael, if you got into a case like that and the kid had a stricture, in a way, the stricture was sort of driving that proximal dilatation. Would you fix the anatomic stricture and see if that suffices, or do the step at the time that you fix the stricture there? That's why you recruit smart partners, because there's not an easy operative decision to make on that. I would argue that the management of these kids is not a one-stop shopping that you're going to fix that kid with your operation. There is nothing wrong with staging. So in this case, Dr. Helmrath is saying if this child with a stricture continues to feed, the bowel. They're going to continue to dilate over time and they're going to have more problems that you have to deal with. And so, maybe the first operation isn't going to be the one that fixes everything for them. And that's why he's saying that it's OK to do things in stage procedures, right? You maybe fix the stricture at first and then if down the road you need to do the step, that might be a consideration, but you don't necessarily have to do it all at once. And there are some important complications to consider when thinking about the step. Procedure. One of the things that we've seen as a complication here is that the kids have done really well with step procedures, but they have continued loss from either they lose protein in the stool or we see recurrent bleeding. Dr. Halmra says ulcers at step lines are not uncommon, and he had many kids that have been transfused for every month for years because of that. And that's an absolute indication to operate. Once you're able to resolve the underlying issue, i.e., remove the. Ulcer, then kids really bounce back after that. They stop losing their protein, their albumin, they stop bleeding, and they're able to grow a lot better. So the approach to the child with chronic blood loss in the step line is very much a surgical consideration. I agree, but I think A, it's an underreported complication, B, it's really difficult to manage in some cases. So Doctor Wales says there's a whole spectrum of findings from these. Ulcers at the staple lines from just having specks of blood in the stool to enough bleeding that the kids might have to be transfused every week and a half. And often these ulcers can recur. My hypothesis is that this is a microbiome problem. I think you have a pro-inflammatory environment. It tends to occur sort of type 2 anatomy, which is sort of a small bowel to colonic remnant in the absence of an intact colon ileocecal valve. So you've got a sig mucosa, which Means more bleeding, and Dr. Wells explains what you see on the pathology. It's non-specific inflammation. There's no vasculitis, there's no viral elements, there's no obvious ischemia, and the management can be super difficult. And teams working on intestinal rehabilitation have tried a lot of things, including enteral omega 3 lipid supplements, bacterial overgrowth management with cycled antibiotics or probiotics, 5ASA, budesonide. Immune modulators like Remicade. We've tried everything, but it sounds like none of those have been the perfect remedy. I totally agree with you that the overgrowth is a pan-inflammation, and that's why GI needs a scope and you need to be present and you need to look at the pattern of inflammation that's occurring. But Doctor Helmrath says that when you really look to see what the issue is, the issue isn't in the bowel with the, with this pan inflammation. The issue is actually in the mesentery. And so that mesenteric fire that Causes inflammation and scarring, creates an obstruction to venous outflow. And what you end up having is venous hypertension along the staple lines. But how does it happen, Ellen? You have all of this inflammation in the mesentery, causes scarring and actually obstructs the venous outflow. So then on the bowel, you'll see enlarged veins and venous hypertension, and even large lymph nodes from lymphatic obstruction. And when you operate. You can see vessels the size of your thumb, and you'll see really big adenopathy because the lymphatics are also obstructed. And it's very important to free up that mesentery from that scar, and it's a dense scar. And Doctor Helma says once you free up that scar, those big vessels come right back to normal. And then the question is, do you have to revise the step? Because I think the venous hypertension is what's leading to the bleeding that we have seen. And Doctor Helmth will typically revise the staple line if this is the issue that He sees. I usually do it always with a hand sewn stitch and just reconnect it back. So the key to me in that situation of bleeding is look at the mesentery. Make sure you free up the mesentery. Don't just look at the bowel. The key point here is that even though the issue seems to be in the bowel, the underlying problem is actually in the mesentery. People say, how do you know you'll find it? It's so obvious if you're looking for it. The question is, when you have Step lines. How do you know how many colleagues here in GI label with methylene blue the ones that it helps. There's a lot of intraoperative decision making and this is one of them. So, if I need to learn one thing from this episode, that will be highlighting the fact that you got to look at the mesentery in these patients. Is it almost an expectation as a part of caring for these patients postoperatively that you're going to have to go back and maybe make res revisions to a stage. Approach. Does that sound about right? First of all, most of the referrals, they've had multiple operations typically before they've come to you. So keep your eye on the horizon, which is what can we do to try to optimize this kid's anatomy. And if you think about that plan, it may not be conducive to just one operation today. You have to set yourself up sometimes planning for the next case. So it sounds like doing things in a staged fashion is totally typical for this patient population. If you read my op notes, I'm only talking to myself, and I'm telling myself, when you're back, this is what you want to do, and orientation and landmarks and what I did. And it's not that I expect to have to always operate again. You know, these kids are going to grow and develop. As they grow and develop, more issues might arise. And Doctor Helmrath has an advice. Try not to be the hero. Try not to do everything, especially the first week in life. Understand biology and physiology and growth is a spectrum, and. Optimize it. You have to be involved with the process. These are families that you need to grow up with. This population is one that the surgeon plays a huge role in, even when they're doing well, because you have to see the progress. So again, very important to have a multidisciplinary team looking at these patients, as Dr. Helmratz and Dr. Well noted, this is a lifelong issue, and so they typically follow these patients for a long time, and that is to be expected. So, in this episode, episode 4 between parts 1 and part 2, we reviewed the surgical management of patients with shortcut, specifically talking about some of the details preoperatively, operatively, and postoperatively for the step procedure. Don't forget, if you didn't already, take a listen to part one. It had a lot of great details in there. As always, don't forget to subscribe to our YouTube channel, leave us a comment on Apple Podcasts or Spotify, wherever you're listening. Download the Stay Current and pediatric surgery app where you can listen to more of this series. I'm Ellen Ancisco. I'm I'm Tom Bash, and this is Stay Current podcasts.
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