Earlier this year, we did some podcasts on anal rectal malformations, but now we're going to talk about some of the problems that we see with anal rectal malformations. So today's podcast is about complications and difficult challenges with anal rectal malformations with Dr. Mark Leavitt. This episode of Stay Current is brought to you by the Center for Colorectal and Pelvic Reconstruction at Nationwide Children's Hospital. Their collaborative team performs more than 1,000 procedures each year for kids from around the world. Visit nationwidechildrens.org forward slash ccpr for more information. Stay Current is a multimedia publication designed to keep healthcare professionals up to date with standards of care and new emerging ideas. This episode is recorded and edited by Todd Ponsky, Nicholas Bruns, and Ian Glenn in partnership with GlobalCastMD and is recorded and produced at Akron Children's Hospital in Akron, Ohio. Welcome to Stay Current in Pediatric Surgery. Today we're going to be talking about complications and difficult situations with anal rectal malformations. And with us we have clearly one of the experts in the field, Dr. Mark Leavitt, who comes to us from Nationwide Children's Hospital. We've worked with Dr. Leavitt and he's been providing us a lot of this content specifically before on Hirschsprung's disease. And now he will be talking to us about some of the difficult situations in anal rectal disease. As we know, he's an expert in this. Mark, thanks for joining us. Hi, Todd. Great to be back. Mark, tell us again your position at Nationwide Children's Hospital. So I am the surgical director here of the Center for Colorectal and Pelvic Reconstruction, which is a four-part center that involves colorectal surgery, urology, gynecology, and GI motility, fully integrated to take care of patients that have one, two, three, or all four of those problems. Great. So ARM, the topic we're about to talk about, is probably the best served by such a center, as there are many patients that need certainly three, but some four of those working groups. Yeah. Well, Mark, let's dig right in. Let me talk to you about a common problem maybe is when a baby is born with an anal rectal malformation, how are some of the ways that if the newborn exam is not accurate, it can lead to future problems? For example, let's talk about a perineal fistula in a male and failure recognized that perineal fistula. What's the problem with that? So you'd be surprised how common these malformations are not properly diagnosed or completely missed in the newborn period. With regard to a male, a baby may very well be passing meconium, and no one will even notice that there's anything wrong with their anal rectal anatomy. And then that kind of patient will present usually in the first year of life, often with severe constipation. And then finally, someone takes a careful look at the anus and finds that, in fact, the hole is too small and it is anterior to the center of the sphincters. And by that time, the rectum and sigmoid have dilated up because the stool has been passing through a very tiny and fistulous orifice, which is not a normal anal or rectal mucosa. And that kid then needs surgery at that time to make a better anoplasty and a proper egress for stool. Of course, the more dramatic case is the missed anorectal malformation that leads to a perforation if the stool doesn't pass satisfactorily, and that can happen as well. There have been patients that have been sent home with no opening at all, meaning no one looked and no one guaranteed that they passed meconium. And, you know, nowadays, the standard is to not even check a rectal temperature, is to check a temperature on the forehead or in the ear, and therefore, you don't have to look. If you don't look, you might not know. Mark, let me ask you a question which may be heresy. We know that children that have a perineal fistula may have constipation. Do we know that relocating that into the sphincters fixes that constipation? No, in fact, it doesn't. It doesn't fix it completely, I would say. It certainly improves the anatomy by making the hole adequately sized and makes the hole lined by mucosa so that it is not this fistulous tract that never grows with the child. So I think it does improve things somewhat. However, they will inherently have some constipation, which needs to be aggressively treated from the outset. I also think that the hole, if it's not centered in the sphincter, actually patients like that, if not operated on, can have some semblance of continence. However, if you really delve deeply, they have loose stool. If they have loose stool, they will soil, and if they do something athletic, they will soil. Because when they squeeze their sphincters, they can't completely close the hole. And that's why I think a proper analplasty is what they need to have done. Okay. When they see you in the clinic, just by eyeballing it, can you tell that this is a perineal fistula? Yeah, that's a very good question. You should be able to tell because you can see the hole. You can see that the hole looks too small. You should check the hole with Hagar dilators. A newborn should be a size 12, and a one-year-old should be a size 15. And also, you normally can see a pinkish ellipse, which is the sphincter mechanism. And in a perineal fistula, you'll find that the hole is in the anterior portion of that ellipse or anterior completely to where that ellipse is. Okay. Sometimes, I'm sorry, sometimes there is also something called a bucket handle, which is a little lifted skin tag that you can actually pass a probe underneath. And that is consistent also with a perineal fistula. You may not see the fistula, but you can be very confident that under that skin tag, there is a fistula. And then you also may see little beads of either meconium, which is black, or mucus, which is white, along the scrotal raffae. And that, too, is consistent with a perineal fistula. Okay. I wonder, you know, it would be great, Mark, if you could get us some pictures of this and we could embed it into this recording and into the text. Happily. That would be great. So let's talk about perineal fistula in a female. This is probably the most confounding thing in pediatric surgery, at least in colorectal pediatric surgery, is how to make this diagnosis correctly. There are many, many patients that are either being missed or being overdiagnosed. And I should talk about both those circumstances. So the miss is very similar to the male, like we discussed. If you see meconium in the diaper and don't look carefully, you can easily miss the fact that the hole where the stool is coming out is not properly sized or properly located. And the criteria that I use is, is the hole too close to the vagina? Namely, is there an inadequate perineal body? Is the hole an adequate size? And I check with Hagar's for that. And is the hole centered in the sphincter or not? And if it's not centered in the sphincter, it's anteriorly located. By definition, it will have too short of a perineal body. That's a perineal fistula. But it is amazing how many times that is missed, first of all. But it's also amazing how many times that a patient is diagnosed with an anorectal malformation. In fact, they are normal. So if that hole is of adequate size and if that hole is in the center of the sphincter, even if that hole is appearing to be a little bit anterior and the perineal body looks a little short, that is a patient that does not need any surgery. They will grow. The perineal body will lengthen. There's nothing to do to improve that patient. One of my professors like to say it's very hard to improve on an asymptomatic patient. So those are the key criteria. And I get referred patients a lot that have been scheduled already for an anoplasty by a pediatric surgeon, and they see me for a second opinion, and I say there's nothing to do. I would not operate on that patient because they've met the criteria. The hole is the right size. There's at least some perineal body, and the hole is properly centered in the sphincter. Yeah. So, in fact, that's happened to me where I've seen patients and really wondered, because they look high to me, but they look like they're in the sphincter muscle, and they look normal size. So that's good to know that those are patients we don't need to do much with. If you're not sure, you can do an examination under anesthesia and stimulate. Right. And confirm or deny that that hole is properly centered within the sphincter. And very frequently, you will find that it is okay. It's properly centered, and there's nothing that needs to be done. So, you know, I have to tell you a funny side story here. I just came back from Peru, and the group from Medical College of Wisconsin, Milwaukee Children's, were in Yantala in the jungle, and there was several children that had issues with previous anorectal malformation surgery. And they didn't have a Pena stimulator. So he took the back wires from a Nokia phone charger that was 12 volts and actually created a Pena stimulator that way. It was actually pretty impressive. Well, that's brilliant. Well, I'll tell you a follow-up to that. When I came to Nationwide, they said, we need to order some more stimulators, the box with the probe that's connected. And they said, we're going to order two, and they each cost $15,000. Is that okay? And I said, you don't need to order those because you can take the anesthesia stimulator, which is $150, and then put these two nice little probes into the stimulator and connect it to little needles, and you get a beautiful sphincteric response. So we got a $150 stimulator instead of a $15,000 stimulator, which worked just as well. Of course, I got no benefit from that $15,000 times two credit, but we use the anesthesia nerve stimulator. You just need a different needle than what they use, which are little calipers. That's a great bit of information. There's actually an article about it in JPS describing how to do that, and it's a group out of Israel that wrote that up. That's wonderful. I love that. But I like the Nokia charger even better. Yeah, that was actually Jorge Godoy from Chile. Sure, I know him. Yeah, he's very innovative. What about the rectourethral fistula? So in such a patient, in the newborn, there's really no anal opening at all. There's no obvious fistula. There may be an anal dimple, but if you really spread that area with your fingers, you'll notice there's no hole and there's no hope for a hole. Some of those babies actually pee meconium, and then that makes the diagnosis. And that's obviously the vast majority of the male patients. They're going to have a rectourethral fistula. It's important to diagnose that. It's important, in my opinion, not to approach that primarily because you just simply don't know where the rectum is. The rectum could be at the bladder, neck, prostatic, or bulbar level. And if you look for it, you're going to find something that is not rectum because if you do a posterior sagittal incision, you'll find something that's midline, white, and shiny, and that might be the urinary tract. If you try laparoscopy, they all go slightly below the peritoneal reflection. You really can't tell whether you're dealing with a bladder, neck, prostatic, or bulbar fistula. So at the moment, those patients are all getting dealt with by, in my opinion safely, by colostomy with distal colostogram. However, for certain patients, and this is exceedingly rare, you might have a cross-table lateral film at the 20th hour or so that shows a very low rectum, and I will approach that one primarily because I want to know if I can know for certain that if I open posterior sagittal, the first thing I'll find is the rectum, then I think you can get a safe operation. Perfect. Okay. What about failure to diagnose a cloaca? I'm sorry. What about failure to diagnose a cloaca? Yeah. Believe it or not, that has happened too. I just, in fact, last week saw a six-month-old who presented at age six months with constipation, and someone finally looked and saw there was no anus, referred the patient to a pediatric surgeon who did a colostomy, who sent the patient to me for the cloaca repair. And, in fact, they have a very standard cloaca with not a hint of an anal opening, and obviously that patient was never examined, and she was very lucky because the cloaca is quite low, and she's been successfully stooling out the cloaca, which is quite remarkable, actually. So, you know, I just think that we need to standardize our newborn exam. I think their lost art of examining the anus and ensuring that someone's putting a rectal thermometer in is an unfortunate change into modern medicine because I think it's harder to miss an anal rectal malformation if someone's put a probe through. Mm-hmm. But that's not happening anymore, so you really need the nurse or nurse practitioner or pediatrician who assesses a newborn to really conscientiously look and make sure that the hole is not just a hole, but it's of adequate size and it seems like it's in the right location. If there's any concern, have a pediatric surgeon take a look. Okay. Okay. This is, you know, there is, people sometimes can get confused when they find a child and they wonder if there's an ambiguous genitalia and there's one opening. It's pretty much always going to be a cloaca. Is that right? Well, I think it's important to know to not misuse that term. Ambiguous genitalia to me means there's clitoromegaly, and it might be consistent with a urogenital sinus with virilization. However, that patient has a completely normal anus. Mm-hmm. So if you're dealing with what appears to be clitoromegaly, and many cloaca patients have large clitorises, but not the kind that are hypertrophied from endocrine stimulation, that patient doesn't have an anus, and that's the big difference. And that patient with no anus and a urogenital sinus, which is essentially a cloaca, Right. has no endocrine problem whatsoever, has two completely normal ovaries. Right. Urogenital sinus by itself as its own entity with normal anus is a unique entity often dealt with by the urologist. Okay. Mark, discuss with me something that I know is very common and I've heard you speak about a lot is the colostomy problems. What mistakes have you seen people make that lead to problems with the colostomy? So I think that operation of a colostomy needs to be taken very seriously, and every pediatric surgeon who deals with newborns, which is the art of our specialty, needs to be really, really good at this because there's a lot of morbidity that's conveyed to a patient with an improperly done colostomy. So the colostomy pitfalls, probably the most common is that the surgeon opened the colostomy too distal in the sigmoid so that the ultimate pull-through is restricted by the location of the colostomy or the location of the mucous fistula. That's extremely common. The other common pitfall, an incompletely diverting loop. Some people claim that their loops are diverting, which I'm sure is true, but some loops are very flat and it's very easy for stool to spill across. I prefer separated stomas so that there can't be a chance of any stool going across, but if stool goes across, it can contaminate the distal segment and lead to urinary tract infections. Now, although there was a recent article saying that UTIs were not more common with loops, but I predict those were really well-done loops, meaning loops that were basically completely diverting. I don't want to take a chance, so I separate the two stomas. The other common error, which doesn't really happen too much anymore, although there are still some parts of the world that are doing transverse colostomies, those are problematic because they can prolapse. But also, if there's a large rectourethral fistula, then the left colon absorbs all the urine. It doesn't come out the mucous fistula. And then the patient can get actually an acidosis related to absorption of urine. In addition, it's very difficult to do a distal colostogram through a transverse colostomy because you have to put in a lot of pressure. And it's very hard to clean all the meconium out in the OR because the entire distal segment is filled with stool. And then they sit there with meconium for all those months. So my preference is essentially to do a very proximal sigmoid colostomy, and that leaves your entire sigmoid loop for the pull-through. And I make that mucous fistula very tiny and flat and separated from the proximal. Nowadays, I would do that with laparoscopy. I would bring the loop out the planned circular hole where the stoma is, divide it, clean out the distal segment, and then underneath the fascia pass the distal segment and open a mucous fistula so you have two openings but no incision between them. Mm-hmm. I see. Okay. So do you find high rates of prolapse when you put them in the incision? Prolapse is related to where in the colon you're choosing to do the colostomy. So if you do a mid-transverse colostomy, both sides could prolapse. Right. If you do a hepatic flexor colostomy, only the distal would prolapse because the right colon is 6 to the right retroperitoneum. If you did a proximal sigmoid colostomy, the only thing that could prolapse would be the distal segment. That's why I make the mucous fistula tiny and flat. The proximal sigmoid will not prolapse because the left colon is fixed to the left retroperitoneum. So it depends on where you bring out your stoma. Ileostomies prolapse all the time because they're free-floating inside the abdomen unless you actually tack them to the anterior abdominal wall. So it has to do with the mobility of the colon proximal to or distal to the open stoma. Okay. All right, Mark. So you're going ahead. You're doing the anoplasty. How do you decide where to put the anoplasty to make sure it's not put in the wrong place? I know we've all seen them be put in the wrong place, especially from your slideshows. Yeah, that's a very good question. And it's surprising how many anoplasties I see that were not placed in the right location. And clearly the surgeon was a good, confident surgeon and does a neat job, but the anus has just simply landed in the wrong place. I actually mark the anoplasty before I make the incision. And I think you can get lost when you're looking at a bunch of jumping muscles from a stimulator. So I will actually draw a circle around that ellipse of where the pinkish ellipse is and where it stimulates on the skin surface before I start. And you can even put a stitch in there if you want, if you're worried about the marker disappearing by the time you're ready for that part. And then you open your posterior sagittal incision or you start your laparoscopy. And then you have a landmark. I think what happens is if people don't do that, then they're open in, let's say, a posterior sagittal incision. And then they see a bunch of muscles jumping around with the stimulator and they choose the wrong place. And those patients need to be redone if they have good potential for bowel control because they have excellent muscles usually, but those muscles are not closing the hole because the hole is not in the center of them. And for that reason, in many of those circumstances, I'll offer them a reoperation to improve their continence potential. Okay. That's interesting. So a lot of look on the outside of the perineum before you start making any incision. Yes. Then you won't get confused once everything is open. I think that's a great tip. And I'm not sure. I actually, I've relied a lot on those, on the stimulated muscles. But you're right. I know you can make yourself think anything when you're looking at them jumping all over the place. I'm sure that's what's happened because really, really good surgeons have put anuses in crazy places. And I think it's because they don't have a sense of what's the center because everything's disrupted once it's open. Yeah. So, Mark, going back preoperatively and doing the distal colostagram, I know you've talked about people misinterpreting that. What are your thoughts on that? Yeah. So the distal colostagram is really an absolutely vital study. And a lot of mistakes are made because of a poorly done study, first of all, and then a misinterpretation of that study. So you really need a radiologist that you work closely with that knows what you need as far as information, as far as what will the test tell you and how will that change your plan surgically. You really want a radiologist that understands that. And the basic question is where is the rectum and how low is it? And is it reachable posterior sagittally or is it better approached laparoscopically? And what is its relationship to the urinary tract? And the common mistake that's made is not enough contrast is given and not enough pressure is given into the distal segment. So you get a false impression that the rectum is high. Or you get a false impression that the rectum is high and that there's no fistula. And if you see a straight line on the bottom of that rectum that corresponds to a line that you could draw from the pubic bone to the coccyx, namely the pubococcygeal line, if you see that flattening of the rectum, you know the radiologist did not give enough contrast, enough pressure. Because you need to overcome the PC line because that's the sphincters. That's where the sphincters are compressing the distal rectum. And very frequently, if you give a little more pressure at that point, you then see a bulging rectum at the bottom and an evidence of a fistula. And that's what you want. If you see that, you'll know exactly where the rectum is. And then you can decide, am I going to attack this posterior sagittally or am I going to attack this laparoscopically? The fistula itself can be at the bladder neck, at the prostatic level, or at the bulbar level. I like to look at the urethra and think of it as a reverse C or let's say an elbow. And I think if the fistula is at the elbow or below, it's a bulbar fistula. And if the fistula is above the elbow, it's a prostatic fistula. And if the fistula is at the bladder neck, it's a bladder neck fistula. And then you know what to plan. And then the other thing I always like to know is not just where the fistula is, but also how does the rectum look? Is it bulbous or is it very tapered? If it's bulbous, it might be reachable posterior sagittally and hard to do laparoscopically because there's a lot of girth to the rectum. Or if it's a tapered rectum, you're better off laparoscopically. You don't want to deal with that posterior sagittally. Yeah. So, Mark, is it possible you could get us photographs of all the different situations you were talking about? I will. I will be happy to do that. An inadequate one and one that's good, one that shows all the different locations. And so it would be helpful to not only look at that ourselves but to show our radiologists. Yeah. I'll be happy to do that. What about when you're doing the operation? Failure to identify the location of the distal rectum. You've mentioned this. You've alluded to this before. And then accidentally making a urologic injury. Can you go over that again? Sure. So if you don't know where the rectum is or you have a false sense of where the rectum is and you open posterior sagittally and you start coming down, staying perfectly in the midline, you will find a whitish, shiny structure. And you'll say, aha, that's the rectum. And you may, in fact, try to mobilize it. And often that's not the rectum at all. It's the bladder neck. So how do you avoid making that? You avoid making that error by knowing exactly where the rectum is. So when you do a distal colostogram, you need to make sure it's a properly done distal colostogram, as we've discussed. Then you know where to look for the rectum. When you open posterior sagittally, you know either the rectum is right under the coccyx. And if you're any lower than that, everything is urinary tract. You need to go right under the coccyx. That's usually where the prostatic one is. It's distal to the coccyx. That's usually where a bulbar is. Or the rectum isn't posterior sagittal at all. You'll never find it through that incision. That's a bladder neck. And then you're better off doing laparoscopy. And I think you're better off doing laparoscopy for the very high prostatics also, particularly with the tapered rectums. But the bulbous prostatics, you can find them right under the coccyx. And I prefer to do that posterior sagittally. And the bulbars are nearly at the perineal skin. And I definitely suggest those be done posterior sagittally and not laparoscopically because it's a lot more work to get to that distal rectum transabdominally than it is posterior sagittally. Are there any tricks intraoperatively if you may have been confused by your colostridgram and you're there to try to make sure you're looking at the right anatomy? Well, I haven't done this, but some people put a little catheter with a balloon in the mucous fistula so they can blow it up with some fluid. Okay. I know some have actually put even a gastroscope and they look for the light. I've never done either of those things, but they both sound like intriguing ideas. I will, if I'm confident or if I feel like it's the rectum, I'll put stitches on either side and open in the midline. And if I'm wrong, I just will close it because that hasn't happened to me. But theoretically, if you stay perfectly in the midline and happen to open into the urinary tract and know that you've done it, you close it. You should really try to avoid that situation, however. The key is a proper distal colostridgram. You should never go to the operating room without knowing exactly what anatomy you're going to expect to find. And the big question is, where is the rectum? Is it the most posterior structure? And where is it the most posterior structure? Okay. That's great advice. And I think probably one of the best things that we've discussed so far. Mark, how do you decide when you should be doing this through a posterior sagittal incision or through laparoscopy? So I plan, I will do one of those based on the distal colostridgram. So if the rectum is at the bulbar level or at the low prosthetic level with sort of a bulge to it, I think those rectums are more easily approached posterior sagittally. If the rectum is at a high prosthetic position and is tapered, sort of narrow at the end of it, not bulging, that is best served by laparoscopy. And then rectums that are higher than that at the bladder neck are certainly best served by laparoscopy. If you try to do laparoscopy for a rectum that's bulging below the peritoneal reflection at the low prosthetic or bulbar level, you're asking for a lot more work that's unnecessary. And if you're timid, you may leave behind what I like to call a remnant of the original fistula, a roof, R-O-O-F. The distal rectum is left behind and that causes trouble later. You're much better off dealing with that kind of rectum that's way below the peritoneal reflection posterior sagittally. The other side of that coin, if you try to approach posterior sagittally, a high rectum, it's very difficult to mobilize and you might injure the urinary tract. And those cases, I think, are best served by doing a laparoscopic approach. Okay. That was a pretty good summary. I think there's a lot of people that wonder which approach to take. And I also would like to say that, you know, people talk about the laparoscopy versus PSARP. And I never look at it that way. I think that laparoscopy replaces laparotomy. It's an elegant dissection from above. But don't give away the advantages of the PSARP. I do a mini PSARP when I do laparoscopy so that I really can safely enter the pelvis, enter through the peritoneal reflection. So I don't make this tiny little incision just where the anus is and blindly pass a trocar. I don't see any advantage of that. There's really nothing that you're cutting if you make the incision a little bit bigger. It's much safer. Plus, once the rectum is pulled through, you can nicely tack that rectum to the posterior edge of the muscle complex and avoid prolapse. Prolapse is very common if you don't do something. And some people hitch it to the pelvis. But I'd rather just do it in the posterior sagittal incision to the muscle complex like you would with a standard PSARP. So I like to call mine a laparoscopic-assisted PSARP. I think that's a better terminology. Yeah. You've said that for years, and I still love the way you put that. And actually, you hit on prolapse. What other tips can you give that may reduce the chances of that happening? So I think putting the rectum in the right location, making sure to close the levators properly, making sure to close the posterior wall to the posterior edge of the muscle complex for at least three or four stitches helps avoid prolapse, not dissecting the rectum more than you really have to. So it just sort of lands right where the anus ought to be and therefore not having to do too much trimming. All of those will avoid prolapse. Prolapse will still occur in about 3% of cases, particularly in those without great muscles. But it certainly shouldn't occur more than that. And it does with those principles are not respected. Okay. And how do you manage it when someone does have a prolapse? So rectal prolapse is a problem for a number of reasons. First of all, it causes bleeding. Second, it causes mucus, both of which are annoying. And third, for a patient who has good potential for bowel control, it can actually inhibit their ability to have bowel control because they can't close the opening because there's a rectal prolapse tissue through it. So even though they have good muscles, they can't close their anus. So when I see a prolapse, I usually deal with it, even if it's basically a prolapse more than about 3 millimeters. And, you know, people talk about ectropium versus prolapse. Basically, it's extra reddish tissue, usually circumferential, but it certainly can be unilateral. And I will trim that. The ideal time to trim that is when they still have their colostomy. Some of them only present after the colostomy is closed because they have constipation at that point, and then it bulges out. If it's a circumferential prolapse, I actually have been trying to do half the circumference in two different ambulatory settings. Families prefer that to a circumferential prolapse that keeps them in the hospital for a few days. And that has worked really, really well. And also what's nice about that is you don't have to dilate the patient because half of the circumference is untouched. They won't stricture. Right. And so you basically dissect it out and trim and then just... Yeah, dissect it out full thickness and then lop it off and then resuture the colonic mucosa to the anal skin. Okay. What about how do you prevent a perineal body dehiscence? So this is also a controversial topic. I'm pretty aggressive in females to really repair... First of all, the key is mobilizing the rectum well. You must get the anterior rectal wall completely separated from the posterior vaginal wall. You have to get to that areolar plane between rectum and vagina. If you don't do that, you will leave your analplasty under some tension. And then that can pull back and then leak into the perineal body space and then dehisc the perineal body. It is the most common cause of reoperation that I do is a female repair in which the perineal body dehiscs. And over time, essentially, the child is left with no perineal body. So first, proper anterior rectal wall mobilization, then a good secure closure of the perineal body with probably even 3-0 suture in a baby. And then 4-0 Vicryl I use on the perineal skin. And then I watch the perineum very, very closely. Traditionally, I have done NPO for seven days on 10% dextrose. I've stopped using Hyper-L for seven days, only if it's more than seven days. Lately, we've been trialing clear liquids only and trying to keep them on clear liquids for a week because I realized that the major problem was hard stool. We didn't want them to pass hard stool. And if you give a kid clear liquids, they won't make hard stool. They may make more stool, but they won't make hard stool. And we've seen some really good results from the perineal body healing adequately without this NPO period for a week, which people are very resistant of. But what I would strongly recommend against is doing that case, feeding the patient a regular diet in a day or two, and then sending them out and then not watching the perineum. Because if they get hard stool, passing through your repair, they're going to split it open. And then they'll have a dehiscence. So one of the advantages of the seven-day NPO period was you got to look at the perineum every day and make sure it's healing properly. Okay. I mean, if you see that it's opening, there's not much you could do at that point. Well, actually, no. If you see it's opening, I actually will take... And this happens maybe one or two cases out of about 200, I can tell you. That's not insignificant. I will take them back to the OR and re-suture the perineal body. Really? You can actually save it by doing that. If you don't know it's happening, by the time you see that kid three or four weeks later, the whole thing is dehiscence and there's nothing you can do. But if you deal with it on day five to eight, you can salvage that situation very well. Okay. I think that's a good... I never would have thought to do that. I would have said, well, that stinks and... No, I would take them back. Okay. Take them back to the OR and re-suture them. Okay. Okay. Mark, what are the problems that can result from laparoscopy? Laparoscopy, I think, causes trouble if you try to dissect a rectum that's too low and either you get there, that too close to the urinary tract or you're too timid and you leave behind the distal rectum, remnant of the original fistula. I think you can cause trouble by the passage of a trocar through the perineal incision. I don't do that. I like to make them more of a posterior sagittal incision. Not a big one, maybe three or four centimeters only, but I think it makes for a much safer passage as you hug the hollow of the sacrum on your way into the pelvis. That's another problem with laparoscopy. Also, I think the high rectums, particularly the bladder neck fistulas, the dissection of the distal rectum is quite challenging to make that reach and have good blood supply. You need to be very cognizant of the fact that you must preserve the IMA because there had been a colostomy before and therefore the connection, the collaterals down the left colic may very well have been disrupted. So that rectum is completely dependent on the IMA. And you need to take very tiny distal vessels along the rectal wall, understanding that the rectum has an excellent intramural blood supply supplied by the IMA. If you take the IMA or you take the branches too close to the aorta, then the rectum is going to die because there's no collateralization down the left colic. Okay. So we talked about laparoscopy. What problems can result from a posterior sagittal incision? Well, I think the biggest problem when you do a posterior sagittal incision is if you're going after a rectum and you don't know where the rectum is. And you open posterior sagittal and you are exploring and you find things. You find the bladder neck, you find the urethra, you find the seminal vesicles, you find the vas deferens, you find an ectopic ureter, you find everything but the distal rectum. And the key to avoid that is to know where the rectum is before you go after it. Okay. Mark, what about a PSARP for a rectum that's too high? Yeah. So a PSARP for a rectum that's too high, you are in danger of seeing things in the midline that are whitish. Right. And you might then bring one of those down. There are some famous cases of pull through bladder neck made into beautiful anoplasties. And post-op, the patient was draining liquid out their anoplasty. And in fact, it was the bladder neck. Right. And I remember seeing you talk about that. And I think now we've hit on this a few times that really one of the most risky or dangerous things that can go wrong is not understanding your distal colostrogram and approaching a PSARP from a rectum that's too high. Yes. Regarding the, let's talk about, just to end this, Mark, let's talk about post-operative soiling in a four-year-old. How do you figure out what's going on and how to treat them? I know that's a common problem. Well, an ARM patient that presents at the age of four with soiling is probably one of the most common patients I see. I think it's almost worth its own discussion of how to manage the soiling ARM patient. But I'll give a stab at it. The first question I ask is, does this patient have potential for bowel control? And if a patient has potential for bowel control, to determine whether a patient has potential for bowel control, I like to look at three factors. One is the original type of malformation. Two is the quality of the sacrum and the calculated sacral ratio. And three is the quality of the spine. And I actually believe those three factors can predict continence. I like to call that the ARM continence index. In fact, we're working out actually numbers associated with that. I usually tell parents that I'll give you a grade in type of malformation, quality of sacrum, and quality of spine. And three A's is a continent patient. And three C's is an incontinent patient. What we don't know is the in-betweens, two A's and a B, or three B's, et cetera. And we're going to come up with that as soon as we put enough of these patients through a data collection scheme. But basically, I say, does that patient have potential for bowel control? So a bulbar fistula with a good sacrum, sacral ratio of one, and a normal spine absolutely should have bowel control. However, a bladder neck fistula with a poor sacrum of sacral ratio of 0.4 and a tethered cord or a myelomeningocele, no chance really of that child having good bowel control. One subjective factor is I look at the perineum. Do they look like they have a good anal wink? Do they have good muscles? But that's very subjective. It's not objective like the other three. Well, a four-year-old wants to be clean. My first step in that patient is to get them clean mechanically. And we use bowel management with enemas for that patient, gain their confidence, and then they're clean and in normal underwear and they're very happy. And for those that have potential for bowel control, based on our assessment of their potential, when they get a little older, a little more mature, we try to switch them over to laxatives and see if they can do well without the enemas at all. And then they have successfully achieved voluntary bowel movements at that point. If they don't succeed, they go back to their enema routine. And if you're finding that you can't get them off enemas, then you can talk about an anti-grade option like a Malone. Okay. And when would you talk about doing a redo pull-through? So if there's a patient that has any potential for bowel control at all and the anatomy is not perfect, namely the anus is improperly located or there's an anal stricture or there's a rectal prolapse or there's a posterior urethral diverticulum, formerly that's its formal name. The new name I like to use is a remnant of the original fistula. If that is there, then those are all indications for a redo procedure. And redoing an anoplasty to get a rectum into the center of the synctor could change a patient to then have potential for bowel control. So if they have absolutely no potential for bowel control, then all they really need is a hole through which to do enemas. But most patients have some potential and I will offer a redo in those cases because I want to give them the best possible anatomy to give them the best possible chance of having bowel control. Okay. And your success on the redos is pretty good when you've narrowed it down to the right patient. Correct. It's very good when you've done that. This has been a very informative discussion because there are so many problems that can happen with anal rectum malformations. And my guess is we could go on for a lot longer because I know that this is one of those things that, as you say, you get one shot to do it right. And if not, there are a lot of things that can go wrong as we talked about today. Yes, I'm very passionate, obviously, as you can tell about this subject. And I really, really want to reduce the need for reoperations. I really want surgeons to understand that there are some key principles that, if respected, can make an enormous difference in avoiding future trouble. I think one of the biggest problems with anal rectum malformations is that if you don't get it right, you don't really know for a few years. Most things in surgery, if you don't do it right, you know right away. Like, if you don't sew a hepatic artery together properly during a liver transplant, the next day, you have a thrombosed artery. If you don't put an anus in the right place, you think you did a perfectly fine operation, the patient goes home, everyone's happy, and only four years later do they come soiling. How are you supposed to, as a surgeon, know what to fix about your technique if your problems are only becoming obvious years later? And I think that's why there is so much morbidity in colorectal. Yep. And I think that's a great summary statement. And as always, I and the rest of the world appreciate you taking the time to go over this with us. And as I said, we'll have hopefully some videos, some photographs, and any other information that you think would help the listeners better understand how to prevent and how to treat and actually know when to refer away these complex patients. So, Mark, I appreciate you spending the time today. Great. Thank you so much for having me. And we'll be talking to you soon again, I'm sure. Thanks again. Very good. We hope you enjoyed this episode of Stay Current in Pediatric Surgery. You can listen, watch, or read all content by downloading the Stay Current in Surgery app. Please send questions or comments to us at staycurrentpodcast at gmail.com. We'll see you next time.
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