Colorectal Quiz Episode 2: When to redo a PSARP

anorectoplasty. It's an incredibly particular procedure and as many of you know, if you don't get it perfect, you might not have the best outcomes. And that kind of separates it from other surgical procedures. And on top of that pressure, a lot of surgeons agree that oftentimes, your first shot might be your only shot to give this patient a good outcome and the right anatomy. So what happens when despite our best efforts, the patient still has issues. Well, then we can talk about reoperating. But it's a difficult subject because how do you know when to reoperate? When's it better to just treat medically as opposed to reoperate? If this brief discussion sparked your memory and now you're thinking about a patient who didn't have a great outcome after their anorectoplasty and you were kind of in this tough decision zone, don't feel bad because even some of the best pediatric colorectal surgeons can sometimes struggle with this. How many patients, Jason, have you seen on laxatives that comes with an anus in the wrong place? How many patients have you seen having had a ceceostomy with perfect anatomy? That's a real frustrating one. Right? Right? A lot. That's Dr. Mark Levit at Children's National Hospital out in DC, and he's talking to his friend Dr. Jason Frisher at Cincinnati Children's Hospital. They're talking about something that frustrates a lot of pediatric colorectal surgeons and just surgeons in general. After you've done an anorectoplasty, when do you pull the trigger and say it's time for a redo, redo to redo. And that's what we're going to talk about today. Anorectal malformations post repair that might need a redo. I'm Rod Gerardo from Cincinnati Children's, and this is the colorectal quiz, episode two. Well, welcome back, everyone. We're really glad you're joining us for our colorectal quizzes. This is going to be in conjunction with a review of a Journal of Pediatric Surgery article, which I'm excited to talk about as well. Um, and really where we're here to talk about today is anorectal malformations and post repair. Patients who are having problems that come to your office at a few years of age and there's issues. And not only are there functional issues, but maybe anatomic issues. And so what we're putting out there, proposing is to redo or not to redo. And now happy New Year to everyone. We missed you. Yes, happy New Year Jason. So um yeah, you set this up really well. These are patients, um, we're going to present two patients similar but different that um have had anorectal malformation repairs in the distant past. They're now more likely age of potty training. The first one is uh seven years old. The second one we'll show you is four years old. And the question is, do they need something done to improve their anatomy both from a practical point of view and from a functional point of view? Namely, improving their anatomy will they develop bowel control. So the first case is this boy. At this point, Dr. Levit pulls up an image of a seven-year-old male child who is status post a anorectoplasty. If you're listening to this podcast in the Stay Current app, go ahead and open the Dropbox and click on the link for the first image and you can see what we're looking at. And um, there's some pretty obvious things that one can see. Uh, obviously there's a rectal prolapse and I think anyone can just look at this image and see that the anoplasty was placed too posteriorly. When we did an electrical stimulation on this patient, the center of the sphincter is in the second photo where the white dot is. Now of importance, the original malformation that was a prostatic fistula and the patient has a tethered cord and has sort of a middle of the road sacral ratio of 0.66. So, what would you do? Well, Mark, maybe we should discuss some of the findings. Yes, there's obvious findings of a prolapsed anoplasty and a a posterior position of the anoplasty. But it also has maybe some other more subtle findings. Like to me, the bottom looks quite flat. And I know or you mentioned in the history that it's a rectoprostatic fistula, patient has a tether cord and a sacral ratio. So how do we put all that information together when discussing with the family trying to prognosticate uh the potential for bowel control? Yeah, I mean I think Jason that's a key question and I think all of us that are taking care of patients with anorectal malformations and I mean every pediatric surgeon out there needs to commit that they know what type of malformation it is anatomically. They know the quality of the sacrum and the quality of the spine even in the newborn period because I can tell you, the family doesn't really care how technically elegant is your anoplasty. What they care about is whether that anoplasty that you make is going to work and is the child going to be clean and a normal underwear and just like all the other kids. So prognosis is key and I think we can tell them a lot. So what type of malformation is it? because the higher it is, the worst the prognosis. And then the sacral ratio, 0.7 or greater usually means normal, connotes normal sphic normal sphincters most likely or close to normal sphincters and good um obviously good good muscle tone and the spine, innervation of that area is probably good. All that was representative by a good sacrum. And then the nerve innervation. So keeping in mind that patients can also come with an associated spinal anomaly. Most commonly tethered cord, but the worst, of course, is a mylomeningaceal and those patients have much more trouble with continents. Todd Ponssky was also on the call, and he caught something that both of them said really quickly and kind of brushed off. You all both looked at this image and said you can tell obviously that this is too too low, too posterior. Without in without a stimulator in the office, what do you look for to immediately tell that the anoplasty was done too low? But basically I'm looking for the anal dimple and um there's like a midline raffe and there seems to be a little raised area um which is where the sphincters are and where the anoplasty ought to be. You know, it's amazingly common to have a mislocated anus. Is it the surgeon either uh misses where the center is if they're doing a laparoscopic pull through or if doing a Psarp, they open in the Psarp incision first. And I think a key pitfall is not do that. Mark the sphincters first, then open the Psarp because then you don't get confused at the end when you're trying to place the anoplasty in the correct location. The next image comes up, this time a female, again with what appears to be a posterior anorectoplasty. If you're in the app, go ahead and click the next image. It looks this is obviously a female, it looks posteriorly located and what my eye is drawn to is the ellipse, the pinkish ellipse, a little bit the white dot shows where it ought to be. The urethra and vagina are normal. So this patient was born with a vestibular fistula. The spine is normal and has an excellent sacrum. So this is a much better prognosis bowel control patient but with a similar anatomic problem and we have to decide whether that patient needs a redo or not. What I'm hearing you say is that visual inspection can usually tell you where this should be, yeah, that the stimulator can confirm maybe, but you usually can tell visually about where this should be. And although you said, so that's one point. And although you said that you should mark this off first before cutting, why are people making these more posterior? How do I prevent that as someone who doesn't have the same experience you do? Yeah, so I think the the answer is that you look for the visual cues as Jason said, the ellipse, the color change, where it's a little bit indented or a little bit of a raised area, how long the perenial body makes makes sense. And of course use the stimulator and the stimulat and before you make any incision of any kind, then the stimulator will show you the center and then you mark that. By the way, I get asked all the time about the stimulator. It's the same electrical stimulator that anesthesia uses anesthesia uses for their train of four. And then there's a connection that you can make um uh that has little pins. Really, really inexpensive and you just have to tell your anesthesiologist not to give skeletal muscle relaxin because it's a little bit weaker than the traditional uh stimulator which was super expensive. The other thing, Mark, I want to mention is that when you're doing reconstructing your malformations, there are patients, the high the quote unquote higher the malformations, let's say a bladder neck fistula in a boy, their sphincter complex isn't always where you think it's going to be. Patients like patients who have a higher malformation such as a bladder neck, sometimes those sphincter complexes are sort of more anterior than you think that you're anticipating it might be. So really use the visual cues and the functional or um findings when using an electrical stimulator to put it where it should be. They pull up a recent JPS article. It's titled assessing the benefit of reoperations in patients who suffer from fecal incontinence after repair of their anorectal malformation. If you're in the app, you can just click on the link and you can read the whole article. You know, what are the benefits of doing this? Because I struggle sometimes before I really delved into pediatric colorectal surgery, I always thought that the original reconstruction was it. And of course we want it to be right the first time, but sometimes a redo is the right thing to do. So let's discuss that. Yeah, so I I just want to uh really give a good shout out to Richard Wood and my other uh partners um in Columbus because uh he really um helped document the data on patients on how they were when they showed up and what happened if you redid them. So really for the first time in the Journal of Pediatric Surgery article that we're talking about here is essentially long-term outcomes in patients who have had reoperations. So let's jump in. First of all, why do patients get redos? The vast majority were mislocation. Uh then came stricture and then came some some less common reasons. Most relevant was a remnant of the original fistula, also known as a roof, rectal prolapse and some others. So how did their quality of life change? And their quality of life improved with a redo. They also had an improved ability to achieve continents. Those patients were ought to be given the best opportunity to have nearly normal anatomy if possible. So in this study, they found that 20% of the patients with a poor sacrum or a poor spine actually developed bowel control after their redo. Now those with good potential, a good sacrum and a good spine, they did extremely well. And now the remainder who did not develop voluntary bowel movements, they were still able to be clean with a bowel management program with enemas or integrade using Alalone. Now the average age of the patients in the study is about three and a half years give or take, kind of raises a question. So Mark, I'm going to play a little devil's advocate here. What is the right age to make these decisions? So I think if you know the anatomy is off, you should do the redo. And actually I think there's an advantage to getting the anatomy right the younger the child is. So if there's a two-year-old and the anus is mislocated or there's a bad prolapse, then I would offer them a redo. And then let them live their life in diapers for a year or two with better anatomy and then see if they can successfully potty train, knowing that if they don't, then they go to bowel management with enemas and may or may not need Alalone. The one nuance is unfortunately, many of these patients present after the age of potty training because they're incontinent. And when they're incontinent, you or I evaluate them for that incontinence and find that the reason why they're incontinent is because they don't have the best operation. Their anus isn't in the right place. They may have great sphincters, but they can't close the anal hole when they squeeze them. So we do the redo at that time. I usually add an Alalone at the same time, just so that they can then learn how to get control with their new anatomy before they really, really take their anatomy out for a ride and stop them alone flushes and try to have voluntary bowel movements. That process may take six to 12 months. Now, the patients that we talked about had obviously misplaced anuses or anuses, but what if it's kind of in between? If you see a patient who has a mislocated anus and it's 50% within the sphincter complex. So half in and half out. Three and a half years old, fecally incontinent. Yeah, I think that's a good question. That's more of a philosophical question. If I was presented with that exact scenario, I would probably redo them and do Alalone and then get them perfectly clean mechanically and then see if they can get develop bowel control. So I think a lot of it depends on the family's family's needs and the age of the patient. I'm sorry, I might be maybe a little bit more conservative and let the child take their car out for a ride first, see how it works. If it drives well, stay with that car. If it's not driving well, then consider the redo. I mean, obviously it's a case by case basis, but I I'm I'm sort of assuming that these patients are coming to us fecally incontinent. Right. So I think I think I think the point you're making, which I think is an excellent one is that if they haven't declared their continents yet because they're not old enough to do so from a behavioral point of view, give them a chance. They may succeed. So to summarize, Dr. Levit and Dr. Frisher in conjunction with this JPS article make a really good argument that those patients who are status post their Psarp but are still having some issues with continents functionally, regardless of their original prognosis group, they might benefit from a redo. They might be able to get continents. And finally, I think this paper, which us as surgeons sometimes maybe skip this point, but I think it's the most important important point is that you improve their quality of life. Yes, it's been awesome as as always. You have anything for me? Um, yeah, so uh, now I'm trying to remember what uh, I just learned a joke from a kid. Well, there was a child that was asking, asking his dad an important question. He said, Dad, where does poo come from? And the father did a Google search and got some education and said, well, it's complicated, you eat and then food goes into the stomach, it's digested, nutrition comes out in the in small intestine, water absorbed in the colon, and then there's poo. And the child thought about that for a minute and said, okay, well then where does Tigger come from? Oh boy. Why didn't you record a a different joke if that one's no good. No, we're keeping it until the next time. So, there you have it. The second episode in our colorectal series with Dr. Levit and Dr. Frisher. I think they put a really good argument for a redo. Sometimes incontinence might just come down to anatomy. I'm Rod Gerardo. Thanks for listening and until next time, remember, knowledge should be free.