Collaborative work: Complex Pediatric Anorectal Malformations 2017

I just want to point out a constant theme. We did an intestinal failure event last year or something like that. And it's the same idea. I, I mean, the panel of people that you had in your team was a collaborative thing as well. Well, you can't take care of patients without, I mean, the full, I mean, I am absolutely convinced, having been in different institutions that I am not the determining factor of the outcome of my patient. It is the environment by which you operate in. So, um, but what you don't realize here because you've all lived here. Is that most hospitals don't have these collaborative teams and uh that's this constant theme that I, I, I may be wrong to say that it may be even taken for granted as an outsider coming in, it's just, I, I am so impressed by how many people are involved in these complex patients. But it, it starts small. It doesn't start with 30 members and, and then support members and staff and, and it starts with 2 or 3. Completely dedicated practitioners and then building a team around that and I think that's the key. You don't need to be at the center where we have 3 surgeons and 5 urologists principle, it's the principle of commitment to. Taking care of that patient, that, that patient population, and then building the resources around you, and you're not going to get all those resources right from the start. But Jason, what people do, what they get a consult, you know, there'll be a patient and I'll say, well, you know, let's get a consult from GI, let's get a nutrition consult. That's not a collaborative team. Those are independent consults. What you guys are doing is actually a true collaborative team and, and that takes, and it takes coordination of time and that's one of the parts I didn't present, but it's in order to get. The 3 or 4 of us in the operating room and then the same clinic appointment. And the same preoperative clinic appointment, it takes real collaboration. So we're in clinic together. We have Wednesdays at 9 a.m. free for an hour to discuss the patients. We have incredible nursing and support team that help us gather this information, but it starts with 2 or 3 people and it slowly grows and then you have a team that's committed to this and passionate about it and trying to make that that patient population better. And that's how it starts. I just want to highlight that, you know, the team that you're seeing here, we are supported by so many other people that aren't here today in front of you, and that is our nurses and our back office people that do the heavy lifting to make sure that we can sit together and discuss a patient. Yeah, it's the back office people are doing the coordination for the ORs, for the clinics, for our conferences, and then the nurses, they're the glue that hold us all together, right. And that's a huge, that's a huge part that's also hard for people to get coordinated at their institutions, right? But you can't go and say I need 5 nurses and 4 project managers and you need 2 or 3 committed clinicians, a nurse, and, and just start, and it takes some extra legwork and I do, you know, we all do. I, I call moms on Saturdays and we do all the work to sort of Make it all come together, but it's, it's slow and it's piecemeal, and we're far from perfect. We're constantly growing. Uh, we just added to our team just this year a physical therapist and a behavioral, uh, medicine team, um, to sort of grow and expand, knowing what our needs are as the program grows. You have more needs and you continue to grow the program and improve the program. But I don't think you could start by saying, I have a team of 20 getting ready to take care of this patient population. You know, sometimes maybe we take it for granted. No, we value what we have. We really treasure this. We don't take it for granted, right, right. Yeah, so no, we really know that what we have here is special and the patients and the families that we're privileged to care for are the beneficiaries. We are always learning from each other too, so that's the other benefit, always. Well, one last patient, I think we have a few minutes to touch on, uh, this patient, Dr. Ramesky is going to share with this patient. So this is a patient that we met shortly after she was adopted. Um, all the family really knew that was that she was born with some sort of a malformation in which she didn't have an anus. Otherwise, the diagnosis was really. Unclear and as anyone who's ever reviewed an international adoption adoption record, you know that it's not always particularly specific and sometimes not truthful. In this case it was just rather vague, but she came to us with a colostomy that was divided in the left lower quadrant that had been created in the newborn period. She had been doing fairly well from what everyone could determine. She was growing well and there were no infections or anything that had been reported. Uh, as part of our initial assessment, we took her to the operating room and here's a picture of what her perineum looked like, you can see that there's a single perineal opening and once you do the doctor breach labial traction, you can confirm that indeed there are no other openings. In there she had a pretty flat appearing bottom you can see from here and then the discoloration in the midline in this location, which would suggest to us that the anal sphincters are going to be there. And indeed when you put a muscle stimulator, you can see fairly good contraction of muscles there as part of the exam in the operating room, we did do a 3D cloacogram study which unfortunately Dr. Patel had to leave. But basically, like Jason alluded to earlier, if there's a hole, put a catheter or a camera in it and fill it with contrast, and we used her her mucous fistula and her common channel as a means to do this study. So basically, and if you could zoom in, you could see that there's a radio opaque marker here that's taped alongside the Foley. There's a Foley catheter that's put into the common channel and then fluid contrast is put in there frequently. Um, the catheter will go directly into the vagina rather than the bladder, and then the other important marker is right here. So we mark where we feel like the anus should be with a radio opaque BB, and then we can kind of tell the distance between. So what you're seeing in this study, and maybe this one shows it the best, is you can see the hash marks here. That's a radio opaque marker on the Foley. Here's the common. Channel and then here's where things start to split off. Obviously in the very way back is the rectum inserting on the lower posterior aspect of the vagina, which is a fairly classic connection in a cloaca. And then up front here is the bladder and you can see the urethra emptying here and then there was about a 2 centimeter approximate urethra. We thought looking At this, that the common channel was somewhere between 2 and 3 centimeters. By the study it was 2.3 centimeters. She had two vaginas and two cervices identified during the vaginoscopy portion. We thought the vaginal length was probably around 4 centimeters after subtracting off the common channel. The bladder looked pretty good, good bladder neck, and like I mentioned, the urethra to the perineum was about 2 centimeters. So this patient also had additional other testing. So she had a renal and pelvic ultrasound. There were 2 kidneys, no hydronephrosis, no hydroculpos, which we wouldn't have expected at this age in a patient who's in good health and has a relatively short common channel. She had an MRI of her spine to look for tethered cord, and that was normal, other than a very small lesion on the fecal sac that was thought to be some sort of small cyst that will be followed with time. Here's a picture of her vaginoscopy. Hard to get all this in one picture, but basically at the top of the screen you're seeing the entrance into the two hemivaginas, and then here's the rectal fistula posteriorly. Could I bring up a question cause I think a lot of the audience is pediatric surgeons and although this patient didn't have a hydrocopos and then we're not going to get to one that did, I think that's one of the areas where pediatric surgeons, um, Have questions and this is the middle of the night phone call that someone gets and what do you do with a patient who isn't making urine, has a hydro has a mass that you assume is a hydrocopos, and maybe Dr. Breach wants to comment a little bit on the neonatal aspects of a cloaca that is obstructed. Sure, I mean, I think one of the important aspects is to be able to have the baby be able to empty urine. And I think when the vagina is filled with fluid, urine, it can prohibit that from happening. It can also put pressure on the ureters. And so as much as I am very interested in emptying the vagina for the vagina's sake, it's really more for kidney health and for the baby to be able to be able to release the urine. So I think one of the things that we have talked about in our team is draining the vagina itself. There have been a number of different approaches to be able to drain urine adequately that ranges from Um, draining urine from the bladder itself, from the vagina. We have typically not had families empty the vagina with a catheter from below into the common channel, but that is certainly a way to empty the urine. We have thought that they're a more durable way of emptying the urine is an important way to do it. Classically, we have had a tube placed through the abdomen. Initially that would be done at the time of the colostomy being created. Um, we have adopted a number of different approaches to that over time, with, uh, including laparoscopy in some patients to be able to place a tube into the vagina. In some patients who may already have a colostomy and not need that surgery, we have engaged our interventional radiology colleagues to place a tube. The concern that I would have as the gynecologist is that often these children have two hemivaginas, so it's important to be able to drain both of those hemivaginas adequately. So at times we have been able to merge the vaginas or make a defect within that septum so that the urine can drain from both sides. So I think that, you know, it's a bit larger discussion about management of hydrocopals, but I would think that sort of take home points are being able to have the urine, be able to be reliably drained until the patient's definitive surgery, and that can be approached a number of different ways either with the initial colostomy surgically. Or if that's already been accomplished, maybe you can engage your interventional radiology colleagues. There are some of our patients who have benefited from draining the urine directly from the bladder with a vesicostomy, and I think that, you know, as we collaborate across the table, as we do in our practice, I would let Dr. Reddy mention that as well, right? Uh, so I agree with what Dr. Breach said that, you know, uh, we do most of the time start off. With a vaginostomy tube to drain, and if we don't see adequate drainage of the upper tract, so if there's persistent hydronephrosis or if there's persistent distention of the bladder, certainly if they're having urinary tract infections, then we know that we have to buff that up a little bit more with an additional drainage and. Uh, it's not easy to catheterize these patients from below, so intermittent catheterization is hit or miss. It also puts a lot of stress on the families, and so, uh, we would resort in that instance if the hydronephrosis has not gotten better. And it's important to understand that it's because there's a lot of pressure on the trigone and where the ureters are entering from that distention of the vagina and the hydrocorpus that's causing that. So, allowing the bladder to empty better with a vesicostomy. The one thing you have to keep in mind though, once you create a vesicostomy, it is going to impair any of the surgery that comes down the road. So you have to be ready to take that vesicostomy down in order to facilitate tension-free mobilization of the pelvic organs and determine if you need that vasostomy again after the surgery has been done. I think one lesson I've learned and watched over the years and from a number of referrals sent to our institution, whatever method you use to drain the, the urine, whether it be through catheterization, vasicostomy, vaginostomy, I think one lesson I learned is you have to check to make sure your system is working, meaning that after you do your procedure or intervention, make sure you're draining what you think you're draining. And so we've had patients that have been catheterizing but not really emptying their hydrocopos and not really draining and therefore causing more and more renal. Effects having downstream renal effects because you're not really draining the problem and so it's important to whatever method you use to study and make sure you're draining what you need and that's just a simple ultrasound and you'll get your answer. Ultrasound is a very powerful tool in the care of these kids. There's no radiation. Most hospitals have it. It's easily applicable to looking at pelvis, bladder, vagina, kidneys. It's an incredibly useful tool. So the whole question to the audience was how would people approach this 2.5 centimeter common channel cloaca and looking at the uh the cloicagram studies can be helpful. Sometimes even with a short common channel, the rectal insertion is very high on the vagina, and in that case, an approach from below wouldn't work, but it looked like 75% of the audience would do a posterior agittal with total urogenital immobilization, and a few people would use laparoscopy here. What we chose to do was go from posterior sagila since we felt like we could do all of the work from below. She had about a 2.5 centimeter common channel length, so the radiology images correlated with what we actually found, and as expected, the rectum was inserting in the posterior vagina. We were able to bring that down to the center of the sphincter complex without moving the mucous fistula. Sometimes the distal segment that's left on the mucous fistula side is too short, and you have to actually close the mucous fistula in order to complete the pull through. We were able to do a total urogenital mobilization. No laparotomy was required. And at the end of the case, number 8 Hagar and number 14, 8, Hagar to the vagina and 14 to the rectum. There's a couple pictures of intraoperatively. So at this point I'd already taken the rectum off of the posterior vagina. So these stitches in the back or on the rectum, which has been tucked up out of place to keep it out of the way during the total urogenital mobilization. Here we have traction sutures on the edge of the vagina, and when you look in here, no that's not a big hole in the vagina. There's a septum there, so you're just seeing light through this side of the vagina. So here's one half of the vagina and here's the other. The stitches up front here on the common channel so you can see the common channel length here and then of course the Foley catheter is in the urethral opening. You can see we've already lifted it off the pubis anteriorly here. You can see there's a space created. So this is with everything being brought down to the perineum. Everything reaches the perineum quite easily in this case and you can see now we've On the roidoplasty, we don't typically take a very good picture of that because it would involve a lot of spreading, and that's the last thing we want to do when we finish all these sutures. Apparently a body reconstructed and then this is part of the creation of the anoplasty. So the anal sphincters were between these. This is the posterior limits of the sphincter, anterior limits of the sphincter, and then there was some posterior sagittal incision to close there. This is the vaginoscopy done post-op. We also resected most of that vaginal septum that you saw, leaving a small amount that was close to the two cervices, and that's what you can see here on the post-op vaginoscopy cervix here and cervix here. This is just a brief slide. So in patients withlika here at Cincinnati Children's, we have a recommended sort of management plan. So as I mentioned earlier, delineating reproductive anatomy as much as possible. This patient actually at the time of her colostomy closure had me race into the OR and do her test menstruation to make sure that both sides are open. So we counsel patients and providers about expectations and then use ultrasound as we can after pubertal development to make sure that things are draining well and there's no concerns about obstruction. So we use those serial ultrasounds until about 6 months after the onset of periods. So this is kind of a good sort of roadmap that you could use for Cloaca patients for the future. And uh I just keep going backwards, sorry, as mentioned, uh, with, so she's now about 6 months post-op, maybe a little longer. Um, she had her colostomy closed about 2 months after everything looked, uh, very good at the time of the closure, and Doctor Beecher was able to come in and assess the malaria. Structures, both sides appear to be patent, so everything was left in place. She's currently on an anal dilation schedule that they're weaning, no stricture, and starting to work on potty training. So there's a lot of things in the future. This child's only not even 3 years old yet. Uh, so a lot of things still to come, but from our standpoint, we're waiting to see if she's going to be continent of stool. She has a normal sacrum, uh, no tethered cord, a relatively short common channel, so we're hopeful that she'll be continent for stool. And from urology, um, again, same things, looking for function and gynecology was addressed. So I think two questions I'd like to ask, and then we'll, I think, wrap things up. One, And I'm asking a whole show to be answered in 2 or 3 minutes, but in this case, we were able to do a total eurogenital mobilization and bring the vagina and urethra into a usable location. If this was a longer. Common channel and or lack of vaginal native vaginal tissue, there are options and we can go into a long talk about that, but Leslie, maybe comment just when we sit on our Wednesday meetings, some of the conversations we have about using small bowel, rectum. Left colon, um. Buckle graphs just sort of briefly. I, I know every case is independent, but we have a few minutes and I think that's a question we always get asked. Yes, that's a great question for the last couple of minutes, right? Um, so I think that we always try to use native vagina for vagina when possible. It's not only hormonally responsive, but has been shown the test of time to function well as a vagina in the future. I think what we really look for is the durability of the tissue, the availability of the tissue, and avoiding any negative impact for the patient for their future when we look for replacements. So I think one of the things that we brought up earlier is we don't want to affect the patient's ability to have a good reservoir for stool. And so looking at rectum is something that we really do in the. Those cases when there's a poor prognosis for the patient for bowel control, tethered cord, poor sacrum, multiple surgeries, those kinds of reasons, we would think that the rectum may be advantageous or in a patient where an abdominal surgery may be less advantageous and the rectum, due to its close geographic proximity, might be an advantage in that particular circumstance. Typically the colon is the tissue of choice that we have used in our experience in our center. It's been durable for future use of the vagina. Any patient that has a vaginal replacement would require a cesarean section for delivery, so that is always discussed with patients and families. But really we're looking at the function in the future for sexual purposes and also for a patent menstrual flow. Small bowel has been utilized, but there are some issues with that, meaning that the pedicle and the blood supply and reaching the pelvis. But as we talked about, there are some patients where that's the best choice because we don't want to spare any colon. We want to keep all colon for colon and not use that for vagina. Lastly, I would say we have been using buckle graft, uh, more commonly, that is, um, a graft of the buccal mucosa, and it seems to act much more like vagina maybe than some of the segments of colon. It has typically been used in patients who are pubertal and that they would be in hospital for about a week's time, really very little mobilization at that time, and then would have a stent in the vagina while the graft takes. We haven't been doing that in our prepubertal patients yet, but there is some opportunity to consider how we will be using buckle grafts. We've used it more for what we call augmentation vaginoplasty in patients who may have strictures later. That's, that's vaginal replacement in 3 minutes. That is phenomenal, and I'm going to ask Pramo a question where, you know, because I always wonder when we're going into these reconstructive cases, when do you decide to divert the urine, meaning when do you put in an SP tube or a suprapubic tube or a vasicostomy versus doing a primary repair. Now in the case presented, it was a 2 centimeter common channel. We expect a good functional outcome immediately post-op. I'm sure we left a fully. catheter in place for a couple of weeks and then went back to the operating room or just did a, a, we probably in this case just pulled the catheter. She did have some voiding dysfunction. Foley was removed. So that's the problem. So when do you decide to divert and let things heal, and when don't you I never know the right answer and that's why I call you. Um, I think that if they have a normal spinal cord, and it's a short common channel that you're most likely going to be OK with either just leaving a urethra catheter. Knowing that if the child's not emptying the bladder, that it's going to be relatively easy for the family to be able to catheterize, and that we can teach them. And I think this question goes back to a bigger question that we, you know, when we are, when we've met as a team, then we are meeting with the family and we are setting. Expectations at each of the toll gates that we're going to pass through in the care of this child. So I think it's really important for anybody that's involved in the care of a child who has ongoing complex surgical needs and um medical management going forward, that you do align what we're able to deliver with the parents' expectations, so that they're ready for what might come next. So when we're talking to the families, we're saying, you know, short common channel, no tethered cord. Most likely going to have a good outcome. We're going to leave a urethral catheter. We're going to take that out. If your child voids, perfect. If not, we're going to check post void residuals, and then we may have to teach you temporarily how to do some in and out cathing. Uh, it might just be temporary edema. It might just be a bladder that's stunned and has some bladder dysfunction that will over time get better. Longer common channels, I think that were a little bit more suspect, and there we would leave a, we would recommend leaving a suprapubic catheter that we can take out the urethral catheter you've left as a stent across the repair, start um clamping the SP tube and seeing, are they able to empty. Um, there is some element of bladder rehab that needs to go in with these children, just like any muscle that hasn't been working correctly before, trying to get this bladder to empty, and one of the things that can really hurt a bladder is incomplete emptying because the bladder muscles just get overstretched, so the cross bridges in those muscle fibers no longer connect and they can't contract. So having a suprapubic catheter that you can have the parents clamp and unclamp to make sure they're not over distending the bladder is critical in that instance. And in a child who's got overtly neurogenic bladder where we know that they're not going to be able to void safely, we're just going to put them in danger with their upper tracts in danger, but we're not ready to do the definitive urological reconstruction there in order to protect the lower tracts in order to prevent people from blindly trying to put a catheter into that urethra that you just spent so much time reconstructing, we would recommend a vascostomy temporarily.