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Welcome to another episode of the Colorectal Quiz. I am Filipe Jalus, Colorectal Research

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Fellow at Children's National Hospital, and today we'll be discussing the Malone procedure.

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Make sure you download the sticker and app to follow along with images and other related

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cases. Welcome back everyone to another episode of the Colorectal Quizzes. You may have thought

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we were hybridating, but no, we were just working. But we're back with some special

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guests and very excited to hear a nice case. I'm so excited to hear today's cases. We are

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joined as always by Dr. Jason Frischer and Mark Levitt. Today our guests are Dr. Jeffrey

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Avanzino from Seattle Children's Hospital and his fellow, Dr. Hira Ahmad. Let's dive

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right in. So today we're going to talk a little bit about Malone appendicostomies and some

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of the challenges that they present to us. And so I'll start off with a case of a 21-year-old

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male who had a history of an perforated anus without fistula. He did not have a history

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of Down syndrome. He's developmentally typical. He had a repair back in 2001. He also had

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the history of a tethered cord that was repaired. And he subsequently underwent a Malone appendicostomy

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as well as Mitrofinov in 2009. For our listeners, a Mitrofinov procedure or appendicovezicostomy

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is a surgical procedure that creates a channel from the bladder to the skin surface, allowing

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the patient to urinate via catheter through a small opening in their lower abdomen. Whereas

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a Malone appendicostomy is a surgical procedure that creates a channel between the abdomen

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and colon to treat fecal incontinence and constipation. These patients Mitrofinov utilized

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a small bowel and was later closed prior to presenting to Dr. Avanzino's office in 2019

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when his appendicostomy started leaking. He had been managed by urology up until that

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point.

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And this appendicostomy just for orientation sake was placed in the umbilicus. And so this

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was leaking and urology at the time was actually doing deflux on these appendicostomies. And

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the original appendicostomy that was placed actually did not have a plication or a valve

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to prevent deflux.

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Deflux is a non-surgical procedure where a sterile biodegradable gel is injected into

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the structural wall allowing fluid to flow in but acting as a valve to prevent black

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flow.

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So despite their initial efforts, the patient came back and one of my partners took the

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patient and did a plication which actually took care of the leakage. And however a year

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later the patient came back after a weight loss of 30 pounds and was leaking again. So

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we took the patient back and replicated the appendix and now the leakage has gone away.

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But when we took the catheter out at four weeks we couldn't replace it. And so now the

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patient had to go back to IR which they were able to place a five centimeter channel length

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AMT button or replace the tube, excuse me, into the appendicostomy.

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If I can just interject for one second. So this is the this is the Malone saga. I'm just

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curious this patient is now 21 and I would venture to say that your management of this

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patient has changed very dramatically had he presented today.

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Can you elaborate Dr. Levin?

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It's very important to know the type of malformation, the quality of the sacrum and the quality

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of the spine and give the patient's family some estimate of the likelihood that they

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will or will not be continent. And I think in a 21 year old who underwent a Malone, well

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they went and underwent a Malone in 2009 so that's 10 years ago, I probably would take

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a moment to say hey does this patient have any potential for continents?

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I see. So you'd want to know if the Malone is really needed before you try to fix a leakage

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you on.

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So do you have any insight into into that? Is this a Malone for life patient or is this

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a patient with some potential?

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Yeah, I mean this this particular patient, they are flush dependent again this patient

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had a tethered cord and is reliant upon enemas to stay clean. It's good to potentially reassess

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if they can be independent of enemas.

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Let's step back to this patient's original procedure. Ten years ago urology did both the

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ACE and the Mitrofanoff. Nowadays and at your institutions this would be done as a joint

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case or at minimum planned together.

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There might be some sharing of the plan and potentially sharing of tissue. So you had

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mentioned that the the Monty was made by from small bowel. It's important to recognize that

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some appendixes and we did handle this topic also on another podcast are shareable and

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some can go from Malone and some can go for the Mitrofanoff. In this case you got the

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whole appendix for the Malone and the Mitrofanoff was done with a small bowel segment.

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Dr. Frischer, what are your thoughts on this original plan of noplication?

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First and foremost what Mark is alluding to is the the shared appendix which is the best

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case scenario when a patient needs both a channel to drain the bladder and a channel

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to give themselves an anti-grade content enema.

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Utilizing a shared appendix has been well studied. About 60 percent of the time there

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is success with using a split appendix. However about 40 percent of the time you just can't

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make it work.

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But just know you need to go into that operation having multiple outs and your outs are basically

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two, right? You could do a Monte Mitrofanoff using small bowel to make that conduit or

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you could do a Neo Malone and use it a sequel or right colon flap to make a channel.

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It's important to remember that a Malone procedure regardless of approach does have morbidity

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associated with it. The most common complication is stricture occurring 17 to 20 percent of

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patients. Less commonly patients can experience leakage like our case study today. The first

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thing you need to know is if they are cleaning themselves out.

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If they're not doing their enemas or they're backed up and the enema is not effective.

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So that was one thing that we did. Another thing you can try doing too is thickening

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them up a little bit so that whatever's coming into the right colon is a little bit thicker.

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And so you could use some you could try some water soluble fiber to see if that helps and

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flush that out with the enema.

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Neither of those maneuvers were successful with this individual. So that's why we did

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the original application and redo application.

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The original management of the leakage though was by a urologist who was using the deflux

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procedure. This was common management for reflux through the ureter and in the late

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2000s was extrapolated to Mitrofanoff's as well.

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It comes down to Pusselli's law. Pusselli's law is the flow of fluid through a tube and

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it's based on the radius to the fourth power and length. So if you take your appendix and

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you have a narrow appendix or a long appendix, the odds of that flow of fluid getting all

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the way to the end is based on those two factors.

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And so a longer appendix sometimes you did imply Kate on those patients.

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I mean I have to say I'm sort of speechless that we're talking about physics with Jason

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Frischer. Frankly what I learned from physics you got to write it out every single step

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prove to yourself that you can do it. And I think that's very appropriate for surgeons

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who are training. You got to put yourself in the position. You got to say can I do this

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by myself? Would I need help? And you got to get to that point. So I learned that life

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lesson the hard way in physics.

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So the longer the appendix the less likely it will leak and you shouldn't need to placate.

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However Dr. Frischer and Levitt did their own study where they did 10 melones in a row

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without placating and five leaked.

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So then we decided they're all getting placated. So we placate them all and I haven't had a

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leakage in a long long time several years. So I think it's a very effective strategy.

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But how do you decide which appendix can be split?

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So basically we came up with these rules which really seem to work. If you have a short and

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stumpy appendix that appendix is best for the melone and the metrophenol ought to be

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made from small bowel. If you have a five to seven centimeter appendix that's not enough

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to share and that ought to go for the metrophenol because long term the metrophenols do much

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better. So therefore you need to make a neo-melone and then if the appendix is seven centimeters

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or greater then it can be split and in my opinion you really need two centimeters minimum

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for the melone and you need five centimeters minimum for the metrophenol.

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Dr. Frischer's research has shown that a neo-melone does just as well as a melone. So if you can

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only use the appendix for one channel it should be the metrophenol because an appendix-based

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metrophenol does much better than a small bowel monty.

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I have a fourth version of that diagram where you're in that five to seven or even four

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to seven centimeter range where I give 90 percent of the appendix to the urologist and

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then I do a sort of extension of the appendiceal stump into the cecum lengthening that channel

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by either using a non-cutting non-pin endo-TA stapler or by hand sewing it with non-absorbable

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braided suture.

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Are there any other questions tips or tricks you would like to share before we move on

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to the next part of this case?

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And I want to ask you, Mark or Jeff, is how do you wrap? Because I've watched my own partners

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wrap two different ways. Sometimes that's dictated by blood supply and sometimes it's

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dictated just by the surgeon. But there's the Nissin type wrap where you could go make a

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window in the mesentery and bring a piece of cecum all the way around wrapping or there's

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just the sort of fold over the appendix 180 wrap from both ends to sort of cover the appendix.

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And so any thoughts on that technique?

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I kind of base that on what the mesentery looks like. You know, if it's a kind of a

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broader base mesentery and you lay the appendix down, that's when I'll kind of make the window

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and do the wrap through the mesentery versus sometimes the mesentery is very adherent,

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like kind of that one vessel and it's not a broad base mesentery. And then those you

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can kind of lay down and wrap the cecum around it.

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The situation where the appendiceal mesentery parallels the appendix, you can basically

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just wrap the cecum around the appendix like a fundoplication. A more typical situation

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where the mesentery is fenestrated and then you ought to go make a window. I usually make

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one window at the very bottom and then placate through that window. Otherwise you crunch

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up the mesentery. So then the other thing to consider is if you're doing a fundoplication

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wrap, you got to see which direction you want to lay your appendix.

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That's a huge little pearl, Mark, because I have seen obstruction at the ileocecal valve

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because of the creation of a Malone. And so paying attention to that, the ileocecal location

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in reference to how you make your Malone and the placation is important.

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Another technique is to imbricate or push the appendix in and pull the cecum up around

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the appendix. Then suture between the appendix and the cecum. It's not commonly used, but

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is another option. As Dr. Levitz said, it's important to know all the possible options

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before going into the case.

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This may be one other thought, especially as we maybe historically have left these longer

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appendices without a placation. How often have you seen patients present with baldness

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around the appendix? And if so, what have we done over time to mitigate for that?

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I have only seen it once confirmed, but I think it's pretty rare.

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I personally have seen it and so I think it happened, but we used to tack the cecum underneath

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the fascia to fixate it.

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I don't tack the cecum anymore, but I definitely make the appendix just what I need it to be.

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So that the cecum is underneath the umbilicus. I don't leave a long stemmed appendix hanging.

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I have seen small bowel volvulize around the appendix, no question, but it's only been

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two or three cases.

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It's also important to recognize that visualizing a floppy cecum with the laparoscope does not

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just mean it may be an easy malone. It also means that this patient could have a volvulus

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and we should be checking the ligament of trites before completing the case.

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Is there anything about how you position the appendix to try to think about how you could

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avoid retrograde catheterization or reflux into the ilium?

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A very interesting question, sir, Avancino. The answer is no. I usually just do the placation

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the way it looks good and I hope for a competent ileocecal valve. When I do a neomalone, I

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try very hard to orient in such a way that the catheter is going to enter into the right

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colon.

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That brings us back to the primary topic of this discussion, troubleshooting malones.

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What are some diagnostic tests that you couldn't do prior to surgery? And what about during

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surgery to make sure you aren't refluxing into the TI?

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If you have a patient that's not doing well and the flushes are not working or if they're

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getting significant symptoms like nausea in particular, you have to do a contrast study

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through your malone and check and see if there's reflux into the terminal ilium because then

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your malone is ineffective.

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Every time I do a placation and every stitch I throw and tie down, I always pass the tube

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to make sure it passes in the direction I want it to do. But again, that's not how it's

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going to lie when it's intracorporeal and you can get in trouble.

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I love that you mentioned that because when we did the replication on the patient I presented,

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the pathotube went just fine.

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I actually don't pass the catheter each time, but I use a 10 French coude as a bougie and

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then I do the placation around that and then of course I check to make sure it casts and

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that usually works. It's usually not one offending stitch if you've left the bougie in for the

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whole time.

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The good news is that most patients don't have any problems post procedure. Only 10

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to 20 percent will actually have issues with their malone. What about developing stenosis?

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Is there any way to mitigate that?

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I think actually that the stenosis rate can be minimized. A couple things that we've done

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over the years is we use a 10 French tube, not 8. We leave it in for a month. We cath

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it twice a day. Many like to have an indwelling tube, so we use a G-tube device, but now they

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make them in 10 French. And I think if that stents the channel for many months, I do believe

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that our incidence of stenosis has gone down because of that.

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If the channel is stenosed, interventional radiology can dilate the tract and place a

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tube to stand it open for months if needed.

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I do have concerns about the tubes we leave in. And I could tell you, yes, you can't get

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a stenosis if you leave a tube in. That's physically impossible. But you're stenting

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that tract open so there's a chance of leakage, right? Your valve no longer is as pertinent

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because you have a stent across the valve. That's one. Two, the bigger problem I've seen

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is when it's leaving tubes in, our amount of prolapse is increased. And I think there's

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a pressure on the appendiceal base pushing up mucosa and the prolapse rate has increased.

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So the common problems with Malone's are leakage, reflux, stenosis, and now prolapse. But it

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sounds like prolapse is most likely secondary to prolonged stenting. What can we learn from

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our urology colleagues?

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I think you're right. That's the etiology. But you are exchanging one problem for another.

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Interestingly, we learned a lot from our urologists on a regular basis. They almost never get

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a stenosis of their metropin off. Why? They cath it every four hours. So what we did is

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we started cathing the Malone tract, if you are a catheter, twice a day, and that has

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reduced the stenosis rate.

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Thank you. That's great insight. Let's get back to our case. You've wrapped the appendix

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twice and currently there is no leakage. But what if it does leak again?

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One thing I would consider, especially after two wraps, is how long is that appendix, right?

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Because is that a problem and do we need to lengthen the channel to help with the leak?

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Yeah. And that's what we did on the second day. The exposed appendix when we went back

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was probably about three to four centimeters. And we wrapped most of that up. So actually

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the exposed appendix was probably a centimeter that we brought up to the umbilicus. Again,

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when we were doing the placation and then after the placation was done, we repass the

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tube and now we can't pass it. So you can see like a bend in the tract that is likely

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the point of obstruction. But likely going to take that patient back and try to see where

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it's kinked.

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This patient you could leave on an indwelling tube. However, he wants to be able to catheterize

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his Malone. What else can we do for this young man?

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Then he needs a Malone plug for a few months and then start, you know, basically keeping

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the Malone plug out for a shorter and shorter time each day and sort of like taper the time

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that the tract is allowed to live alone.

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Another potential problem is that you can't visualize the Malone axis. How would you troubleshoot

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that?

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I took a Lone Star ring and Lone Star pins and I put them in the umbilicus to get incredible

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exposure to the umbilicus. And then we saw the hole and then we got the catheter in the

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tiny little hole. It's not so easy to blind puncture a Malone tract, but you have a very

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thin appendix underneath it and it's pretty easy to go into the sidewall.

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So myself and some of the interventional radiologists here have become very skilled at this and

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maybe a few tricks to share as well. One, probably many of them do this where we blow

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up a balloon on a Foley catheter, occlude the umbilicus and inject dye to see if there's

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a little pinhole tract that you could find. Because you're now not allowing that contrast

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to leak out of the umbilicus and you get a pressurized system in and inject dye and it'll

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find any micro hole that's still there.

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That's really interesting. Isn't there a way to use ultrasound as well?

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You use ultrasound just like you'd find an appendix. We know where it's going to be around

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the umbilicus and then we needle, localize the lumen of the appendix, ultrasound guided,

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and we've rescued a few Malones that way as well.

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That's a really neat technique. I bet the patients are grateful when you've reestablished

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access.

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Hiro, do you have any questions for our experts today?

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Have you ever seen appendicitis in a Malone?

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It's impossible.

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Well, unless the hole closes.

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Unless the hole closes. Great question. We get asked this all the time. In panther physiology,

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there's no obstruction. You can't get an obstructed appendix. Now I have once gone in to do a Malone

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and the patient had appendicitis. So we aborted the procedure, treated the patient medically

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and three months later went in and did a Malone.

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The appendix is pretty important to save in patients who may need a Malone or Mitrofinov

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in the future. Which patients are you less likely to remove the appendix during your

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laths or appendicitis?

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We do not take out the appendix in a Hirschkrum or ARM patient or a kid with a spine issue.

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Or at Absinth Sacrum. There's a whole bunch of spina bifida. No one is obligating you

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to take out the appendix. And I will tell you in South Africa, they never take out the

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appendix as part of a laths. It's just not done. It's a United States thing.

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It's important to know the basics of pediatric surgery, the patient's anatomy and potential

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needs in the future. Hiro, did you have any other questions?

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When you do a Malone, do you always send your appendix to pathology?

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Another good question. I do. It's thanks to our pathologists that insisted. I asked them

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if we need to send the tip of the appendix and they insisted. And guess what? Three months

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later, one of them had a carcinoid.

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Doroendocrine tumor. I just saw the patient today from doing an appendectomy, but it's

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volatile tissue that can carry this tumor. So I think you have to send it to make sure

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there's no tumor there.

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This was such a great discussion. In summary, Malones are great for patients who need a

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mechanical assistance with their bowel clearing, such as those with continence issues who can't

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take medication, for functional constipation patients with colonic dysmotility, ARM patients

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who have no potential for continence and spinal bifida patients who may also need a Mitrofonov.

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We were able to review some key technical points and how to manage key complications

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when they arise. Thank you all for joining us for this episode of the Colorectal Quiz.

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And remember to download the Stay Current app from the Apple App Store or the Google

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Play Store to check out the images and algorithms we discussed in this episode. Additionally,

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remember to follow us on social media and check out our YouTube channel for more pediatric

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surgery content.