Colorectal Quiz: Episode 43

Welcome to another episode of the Colorectal Quiz. I am Philippa Jalus, Colorectal Research Fellow at Children's National Hospital, and today we'll be discussing the Malone procedure. Make sure you download the sticker and app to follow along with images and other related cases. Welcome back everyone to another episode of the Colorectal Quizzes. You may have thought we were hibernating, but no, we were just working. But we're back with some special guests and very excited to hear a nice case. I'm so excited to hear today's cases. We are joined as always by Dr. Jason Frischer and Mark Leavitt. Today, our guests are Dr. Jeffrey Avanzino from Seattle Children's Hospital and his fellow Dr. Hira Ahmad. Let's dive right in. So today we're going to talk a little bit about Malone appendicostomies and some of the challenges that they present to us. And so I'll start off with a case of a 21-year-old male who had a history of anus without fistula. He does not have a history Down syndrome. He's developmentally typical. He had a repair back in 2001. He also has a history of a tethered cord that was repaired. And he subsequently underwent a Malone appendicostomy as well as Mitrofinov in 2009. For our listeners, a Mitrofinov procedure or appendicovisicostomy is a surgical procedure that creates a channel from the bladder to the skin surface, allowing the patients to urinate via catheter through a small opening in their lower abdomen. Whereas a Malone appendicostomy is a surgical procedure that creates a channel between the abdomen and colon to treat fecal incontinence and constipation. These patients, Mitrofinov utilized a small bowel and was later closed prior to presenting to Dr. Avanzino's office in 2019 when his appendicostomy started leaking. He had been managed by urology up until that point. And this appendicostomy, just for orientation sake, was placed in the embolicus. And so this was leaking and urology at the time was actually doing deflux on these appendicostomies. And the original appendicostomy that was placed actually did not have a plication or a valve to prevent reflux. The flux is a non-surgical procedure where a sterile biodegradable gel is injected into the structural wall, allowing fluid to flow in, but acting as a valve to prevent black flow. So despite their initial efforts, the patient came back and one of my partners took the patient and did a plication, which actually took care of the leakage. And however, a year later, the patient came back after a weight loss of 30 pounds and was leaking again. So we took the patient back and replicated the appendix. And now the leakage has gone away. But when we took the catheter out at four weeks, we couldn't replace it. And so now the patient had to go back to IR, which they're able to place a five centimeter channel length AMT button or replace a tube, excuse me, into the appendicostomy. If I can just interject for one second. So this is the, this is the Malone saga. I'm just curious. This patient is now 21. And I would venture to say that your management of this patient has changed very dramatically had he presented today. Can you elaborate Dr. Levitt? It's very important to know the type of malformation, the quality of the sacrum, and the quality of the spine and give the patient's family some estimate of the likelihood that they will or not be continent. And I think in a 21 year old who underwent a Malone, well, they went underwent a Malone in 2009. So that's 10 years ago. I probably would take a moment to say, Hey, does this patient have any potential for continence? I see. So you'd want to know if the Malone is really needed before you try to fix a leaky one. So do you have any insight into, into that? Is this a Malone for life patient or is this a patient with some potential? Yeah. I mean, this, this particular patient, they are flush dependent. Again, this patient had a tethered cord and is reliant upon enemas to stay clean. It's good to potentially reassess if they can be independent of enemas. Let's step back to this patient's original procedure. 10 years ago, urology did both the ACE and the Mitrofenov. Nowadays and at your institutions, this would be done as a joint case or at minimum plan together. There might be some sharing of, of the plan and potentially sharing of tissue. So you had mentioned that the, um, um, the Monty was made by, from small bowel. It's important to recognize that some appendixes, and we did handle this topic also on another podcast are shareable and some can go from Malone and some can go for the Mitrofenov. In this case, you got the whole appendix for the Malone and the Mitrofenov was done with a small bowel segment. Dr. Frischer, what are your thoughts on this original plan of no plication? First and foremost, what Mark is alluding to is the, the shared appendix, which is the best case scenario when a patient needs both a channel to drain the bladder and a channel to give themselves a antigrade content enema. Utilizing a shared appendix has been well studied. About 60% of the time, there is success with using a split appendix. However, about 40% of the time, you just can't make it work. But just know you need to go into that operation having multiple outs and your outs are basically two, right? You could do a Monty Mitrofenov using small bowel to make that conduit, or you could do a Neo Malone and use it, a sequel or right colon flap to make a channel. It's important to remember that a Malone procedure, regardless of approach, does have morbidity associated with it. The most common complication is stricture occurring in 17 to 20% of patients. Less commonly, patients can experience leakage, like our case study today. The first thing you need to know is if they are cleaning themselves out. If they're not doing their enemas or they're backed up and the enemas are not effective. So that was one thing that we did. Another thing you can try doing too is thickening them up a little bit so that whatever's coming into the right colon is a little bit thicker. And so you could use some, you could try some water-soluble fiber to see if that helps and then flush that out with the enema. Neither of those maneuvers were successful with this individual. So that's why we did the original plication and redo plication. The original management of the leakage though was by a urologist who was using the deflux procedure. This was common management for reflux through the ureter and in the late 2000s was extrapolated to mitrafenovs as well. It comes down to Pusselli's law, right? Pusselli's law is the flow of fluid through a tube and it's based on the radius to the fourth power and length. So if you take your appendix and you have a narrow appendix or a long appendix, the odds of that flow of fluid getting all the way to the end is based on those two factors. And so a longer appendix, sometimes you didn't plicate on those patients. I mean, I have to say, I'm sort of speechless that we're talking about physics with Jason Frischer. Frankly, what I learned from physics, you got to write it out every single step, prove to yourself that you can do it. And I think that's very appropriate for surgeons who are training. You've got to put yourself in the position. You got to say, can I do this by myself? Would I need help? And you got to get to that point. So I learned that life lesson the hard way in physics. So the longer the appendix, the less likely it will leak and you shouldn't need to plicate. However, Dr. Frischer and Levitt did their own study where they did 10 malones in a row without plicating and five leaked. So then we decided they're all getting plicated. So we plicate them all. And I haven't had a leakage in a long, long time, several years. So I think it's a very effective strategy. But how do you decide which appendix can be split? So basically we came up with these rules, which really seem to work. If you have a short and stumpy appendix, that appendix is best for the malone and the metrophonov ought to be made from small bowel. If you have a five to seven centimeter appendix, that's not enough to share. And that ought to go for the metrophonov because long term, the metrophonovs do much better. So therefore you need to make a neo malone. And then if the appendix is seven centimeters or greater, then it can be split. And in my opinion, you really need two centimeters minimum for the malone and you need five centimeters minimum for the metrophonov. Dr. Frischer's research has shown that a neo malone does just as well as a malone. So if you can only use the appendix for one channel, it should be the metrophonov because an appendix-based metrophonov does much better than a small bowel monty. I have a fourth version of that diagram where you're in that five to seven or even four to seven centimeter range where I give 90% of the appendix to the urologist. And then I do a sort of extension of the appendiceal stump into the cecum, lengthening that channel by either using a non-cutting, non-pin endo TA stapler or by hand sewing it with non-absorbable braided suture. Are there any other questions, tips or tricks you would like to share before we move on to the next part of this case? Jeff is how do you wrap? Because I've watched my own partners wrap two different ways. Sometimes that's dictated by blood supply and sometimes it's dictated just by the surgeon, but there's the Nissen type wrap where you could go make a window in the mesentery and bring a piece of cecum all the way around wrapping. Or there's just the sort of fold over the appendix 180 wrap from both ends to sort of cover the appendix. And so any thoughts on that technique? I kind of base that on what the mesentery looks like. You know, if it's a kind of a broader base mesentery and you lay the appendix down, that's when I'll kind of make the window and do the wrap through the mesentery versus sometimes the mesentery is very adherent. Like kind of that one vessel and it's not a broad-based mesentery and then those you can kind of lay down and wrap the cecum around it. The situation where the appendiceal mesentery parallels the appendix, you can basically just wrap the cecum around the appendix like a fundoplication. A more typical situation where the mesentery is fenestrated and then you ought to go make a window, I usually make one window at the very bottom and then plicate through that window. Otherwise you crunch up the mesentery. So then the other thing to consider is if you're doing a fundoplication wrap, you got to see which direction you want to lay your appendix. That's a huge little pearl, Mark, because I have seen obstruction at the ileocecal valve because of the creation of a Malone. So paying attention to the ileocecal location in reference to how you make your Malone and the plication is important. Another technique is to imbricate or push the appendix in and pull the cecum up around the appendix. Then suture between the appendix and the cecum. It's not commonly used, but it's another option. As Dr. Levitt said, it's important to know all the possible options before going into the case. Just maybe one other thought, especially as we maybe historically have left these longer appendices without a plication. How often have you seen patients present with spalduos around the appendix? And if so, what have we done over time to mitigate for that? So I have only seen it once confirmed, but I think it's pretty rare. I personally have seen it. And so I think it happened, but we used to tack the cecum underneath the fascia to fixate it. I don't tack the cecum anymore, but I definitely make the appendix just what I need it to be so that the cecum is underneath the umbilicus. I don't leave a long stemmed appendix hanging. I have seen small bowel volvulize around the appendix, no question, but it's only been two or three cases. It's also important to recognize that visualizing a floppy cecum with the laparoscope does not just mean it may be an easy malone. It also means that this patient could have a volvulus and we should be checking the alignment of trites before completing the case. Is there anything about how you position the appendix to try to think about how you could avoid retrograde catheterization or reflux into the ilium? A very interesting question, Sir Avincino. The answer is no. I usually just do the plication the way it looks good. And I hope for a competent iliosecal valve. When I do a neomalone, I try very hard to orient in such a way that the catheter is going to enter into the right colon. That brings us back to the primary topic of this discussion, troubleshooting malones. What are some diagnostic tests that you couldn't do prior to surgery? And what about during surgery to make sure you aren't refluxing into the TI? If you have a patient that's not doing well and the flushes are not working, or if they're getting significant symptoms like nausea in particular, you have to do a contrast study through your malone and check and see if there's reflux into the terminal ilium because your malone is ineffective. Every time I do a plication and every stitch I throw and tie down, I always pass the tube to make sure it passes in the direction I want it to do. But again, that's not how the body's going to, how it's going to lie when it's intracorporeal and you can get in trouble. I love that you mentioned that because when we did the replication on the patient I presented, I passed the tube and it went just fine. I actually don't pass the catheter each time, but we use a 10 French coup d'et as a bougie and then I do the plication around that and then of course I check to make sure it casts and that usually works. It's usually not one offending stitch if you've left the bougie in for the whole time. The good news is that most patients don't have any problems post-procedure. Only 10 to 20% will actually have issues with their malone. What about developing stenosis? Is there any way to mitigate that? I think actually that the stenosis rate can be minimized. A couple of things that we've done over the years is we use a 10 French tube, not eight. We leave it in for a month. We cath it twice a day. Many like to have an indwelling tube. So we use a G-tube device, but now they make them in 10 French. And I think if that stents the channel for many months, I do believe that our incidence of stenosis has gone down because of that. If the canal is stenosed, interventional radiology can dilate the tract and place a tube to stand it open for months if needed. I do have concerns about the tubes we leave in. And I could tell you, yes, you can't get a stenosis if you leave a tube in. That's physically impossible. But you're stenting that tract open. So there's a chance of leakage, right? Your valve no longer is as pertinent because you have a stent across the valve. That's one. Two, the bigger problem I've seen is when it's leaving tubes in, our amount of prolapse has increased. And I think there's a pressure on the appendiceal base pushing up mucosa and the prolapse rate has increased. So the common problems with Malone's are leakage, reflux, stenosis, and now prolapse. But it sounds like prolapse is most likely secondary to prolonged stenting. What can we learn from our urology colleagues? I think you're right. That's the etiology. But you are exchanging one problem for another. Interestingly, we learned a lot from our urologists on a regular basis. They almost never get a stenosis of their metrophenol. Why? They cath it every four hours. So what we did is we started cathing the Malone tract. If you are a catheter twice a day, and that has reduced the stenosis rate. Thank you. That's great insight. Let's get back to our case. You've wrapped your appendix twice, and currently there is no leakage. But what if it does leak again? One thing I would consider, especially after two wraps, is how long is that appendix, right? Because is that a problem? And do we need to lengthen the channel to help with the leak? Yeah. And that's what we did on the second. The exposed appendix, when we went backwards, was probably about three to four centimeters. And we wrapped most of that up. So actually, the exposed appendix was probably a centimeter that we brought up to the umbilicus. Again, when we were both doing the plication, and then after the plication was done, we repassed the tube. And now we can't pass it. So you could see like a bend in the tract that is likely the point of obstruction. We're likely going to take that patient back and try to see where it's kinked. So for this patient, you could leave an indwelling tube. However, he wants to be able to catheterize his malone. What else can we do for this young man? Then he needs a malone plug for a few months and then start, you know, basically keeping the malone plug out for a shorter and shorter time each day. And sort of like taper the time that the tract is allowed to live alone. Another potential problem is that you can't visualize the malone access. How would you troubleshoot that? I took a Lone Star ring and Lone Star pins, and I put them in the umbilicus to get incredible exposure to the umbilicus. And then we saw the hole. And then we got a catheter in the tiny little hole. So it's not so easy to blind puncture a malone tract, but you have a very thin appendix underneath it. And it's pretty easy to go into the sidewall. So myself and some of the interventional radiologists here have become very skilled at this and maybe a few tricks to share as well. One, probably many of them do this, where we blow up a balloon on a Foley catheter, occlude the umbilicus, and inject dye to see if there's a little pinhole tract that you could find. Because you're now not allowing that contrast to leak out of the umbilicus. And you get a pressurized system in and inject dye, and it'll find any micro hole that's still there. That's really interesting. Isn't there a way to use ultrasound as well? You use ultrasound, just like you'd find an appendix. We know where it's going to be, around the umbilicus. And then we needle, localize the lumen of the appendix, ultrasound guided, and we've rescued a few malones that way as well. That's a really neat technique. I bet the patients are grateful when you've reestablished access. Hira, do you have any questions for our experts today? Have you ever seen appendicitis in a malone? It's impossible. Well, unless the hole closes. Unless the hole closes. Great question. We get asked this all the time. In pathophysiology, there's no obstruction. You can't get an obstructed appendix. Now, I have once gone in to do a malone, and the patient had appendicitis. So we aborted the procedure, treated the patient medically, and three months later went in and did a malone. The appendix is pretty important to save in patients who may need a malone or mitrafenov in the future. Which patients are you less likely to remove the appendix during your lads or appendicitis? We do not take out the appendix in a first crones or ARM patient or a kid with spine issues. Or an absent sacrum. There's a whole bunch of spina bifida. No one is obligating you to take out the appendix. And I will tell you, in South Africa, they never take out the appendix as part of a lads. It's just not done. It's a United States thing. It's important to know the basics of pediatric surgery, the patient's anatomy, and potential needs in the future. Hira, did you have any other question? When you do a malone, do you always send your appendix to pathology? Oh, another good question. I do. It's thanks to our pathologists that insisted. I asked them if we need to send the tip of the appendix, and they insisted. And guess what? Three months later, one of them had a carcinoid. A neuroendocrine tumor. I just saw the patient today from doing an appendectomy, but it's volatile tissue that can carry this tumor. So I think you have to send it to make sure there's no tumor there. This was such a great discussion. In summary, malones are great for patients who need a mechanical assistance with their bowel clearing, such as those with continence issues who can't take medication, for functional constipation patients with colonic dysmotility, ARM patients who have no potential for continence, and spina bifida patients who may also need a mitrofenov. We were able to review some key technical points and how to manage key complications when they arise. Thank you all for joining us for this episode of the Colorectal Quiz, and remember to download the Stay Current app from the Apple App Store or the Google Play Store to check out the images and algorithms we discussed in this episode. Additionally, remember to follow us on social media and check out our YouTube channel for more pediatric surgery content.