Welcome to another episode of the Colorectal Quiz. I am Filipe 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 hybridating, 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 Levitt. 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 an perforated anus without fistula. He did not have a history
of Down syndrome. He's developmentally typical. He had a repair back in 2001. He also had
the 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 appendicovezicostomy
is a surgical procedure that creates a channel from the bladder to the skin surface, allowing
the patient 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 umbilicus. 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 deflux.
Deflux 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 were able to place a five centimeter channel length
AMT button or replace the 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. Levin?
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 will not be continent. And I think in a 21 year old who underwent a Malone, well
they went and 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 continents?
I see. So you'd want to know if the Malone is really needed before you try to fix a leakage
you on.
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. Ten years ago urology did both the
ACE and the Mitrofanoff. Nowadays and at your institutions this would be done as a joint
case or at minimum planned together.
There might be some sharing of the plan and potentially sharing of tissue. So you had
mentioned that the 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 Mitrofanoff. In this case you got the
whole appendix for the Malone and the Mitrofanoff was done with a small bowel segment.
Dr. Frischer, what are your thoughts on this original plan of noplication?
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 an anti-grade content enema.
Utilizing a shared appendix has been well studied. About 60 percent of the time there
is success with using a split appendix. However about 40 percent 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 Monte Mitrofanoff 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 17 to 20 percent 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 enema is 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
flush that out with the enema.
Neither of those maneuvers were successful with this individual. So that's why we did
the original application and redo application.
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 Mitrofanoff's as well.
It comes down to Pusselli's law. 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 did imply Kate 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 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 placate.
However Dr. Frischer and Levitt did their own study where they did 10 melones in a row
without placating and five leaked.
So then we decided they're all getting placated. So we placate 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 melone and the metrophenol 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 metrophenol because long term the metrophenols do much
better. So therefore you need to make a neo-melone 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 melone and you need five centimeters minimum for the metrophenol.
Dr. Frischer's research has shown that a neo-melone does just as well as a melone. So if you can
only use the appendix for one channel it should be the metrophenol because an appendix-based
metrophenol 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 percent 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?
And I want to ask you, Mark or 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 Nissin 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 base 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 placate 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. And so paying attention to that, the ileocecal location
in reference to how you make your Malone and the placation 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
is another option. As Dr. Levitz said, it's important to know all the possible options
before going into the case.
This may be one other thought, especially as we maybe historically have left these longer
appendices without a placation. How often have you seen patients present with baldness
around the appendix? And if so, what have we done over time to mitigate for that?
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 ligament 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, Avancino. The answer is no. I usually just do the placation
the way it looks good and I hope for a competent ileocecal 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 then
your malone is ineffective.
Every time I do a placation 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 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,
the pathotube went just fine.
I actually don't pass the catheter each time, but I use a 10 French coude as a bougie and
then I do the placation 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 percent 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 things that we've done
over the years is we use a 10 French tube, not 8. 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 channel 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 is 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 metropin off. 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 the 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 day. The exposed appendix when we went back
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 doing the placation and then after the placation was done, we repass the
tube and now we can't pass it. So you can see like a bend in the tract that is likely
the point of obstruction. But likely going to take that patient back and try to see where
it's kinked.
This patient you could leave on 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 axis. 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 the catheter in the
tiny little hole. 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.
Hiro, 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 panther physiology,
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 Mitrofinov
in the future. Which patients are you less likely to remove the appendix during your
laths or appendicitis?
We do not take out the appendix in a Hirschkrum or ARM patient or a kid with a spine issue.
Or at Absinth 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 laths. 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. Hiro, did you have any other questions?
When you do a Malone, do you always send your appendix to pathology?
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.
Doroendocrine 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 spinal bifida patients who may also need a Mitrofonov.
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.
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