DrBeen Medical Lectures: Dr. Marc Levitt, MD Discusses Hirschsprung Disease

All, this is Doctor Moveen Sayed from Doctor Bean.com. Welcome to one more show. So today we have once more a star with us, a rock star with us. He is one of the top. Surgeons for pelvic surgery and colorectal reconstruction surgery. It is an area where actually once a child is sick with the, for example, if it is in the children, the life becomes pretty difficult. I had a friend whose child was suffering from Hirshprung, and it was a difficult thing for the whole family. So, um, I am pretty excited to have Doctor Mark A. Levitt with us today to discuss about Hirshprung. So, uh, Mark, welcome. Thank you so much for the invitation. It's great to be here. So tell us a little bit about yourself, your work, and your hospital, and uh what is your day to day look like? So I'm a surgeon, a pediatric surgeon. I take care of children up until the age of 21. I work at Children's National Hospital, Washington, DC. And I run a program uh for colorectal and pelvic reconstruction, which essentially means that we care for patients that have congenital, mostly congenital problems of the colon and rectum. So, patients can be born with a variety of anatomic problems where their rectum does not form well and their gynecologic and neurologic systems don't form well. And what we'll be talking about tonight is the anatomy is there, but the physiology of that segment of the colon doesn't work, because it's missing nerves, and therefore it doesn't function, and then the patient can't uh eliminate stool, and of course, then can't eat or be nourished. So, in both of those scenarios, the patients need to be intervened on, um, in the first couple of days of life, and then they have problems uh down the road. That need to be managed by a team of um colorectal experts in collaboration with urology, gynecology, and gastroenterology, and that's the program that I run, that's the career I've been committed to for the last 25 years. So once again, welcome. Uh, thank you very much. Actually, our ambience of our neighborhood, the Doctor Bean medical lectures, is increased with your presence here. You are the chief of your department. You are one of the rarer surgeons who are in this area, and you are one of the top surgeons of the country and I would suspect in the world as well. So once again, thank you very much. It is an honor to have you with us. Uh, how do we want to start? Should I share my screen and uh we go from there? Sure. So for the audience here, here are the References. So this is the hospital. The links are in the description. So this is Children's National Hospital Pediatric, Colorectal and Pelvic reconstruction. That is the department that Dr. Mark Levitt heads. This is the biography of Dr. Mark Levitt, and that is in the description as well. And of course I would remiss if I do not present this to Dr. Bean.com. So with this, let's start. So here we are. Great. So I'm gonna give you an overview of this um rare condition called Hirschrung's disease. Um, it's not so rare when it comes to congenital problems. It occurs in about 1 in 5000 live births. Um, and from a congenital point of view, that's relatively common. What we're gonna try to discuss are the, is the diagnosis of the condition. Of Hirschsprung's disease, we want you to be able to recognize the key signs of the disease and other conditions that could mimic it. We want you to be able to describe the actual diagnostic steps to confirm, um, what it, whether or not it's Hirschprung's disease, understand the surgical treatments that are required, and then recognize the long term outcomes and concerns and how to treat those after the patient's had surgery. Well, let's talk about the newborn. Um, in almost every single case, there is no suspicion whatsoever that there's anything wrong with this newborn, and a baby comes out usually at term. And then the problems ensue when the baby simply cannot pass stool, uh, not infrequently, they do not pass their meconium in the 1st 48 hours of life sometimes, and they are distended, their abdomen is distended, and, and then you recognize there's a problem. Um, 90% of patients are diagnosed in the first couple of months of life, the vast majority in the first week or so of life, and this, um, picture that you can see. Demonstrates the narrowed segment at the bottom is the area where the colon is missing ganglion cells, they haven't migrated to that location, and without ganglion cells, you cannot relax the colon, and therefore it stays squeezed, and therefore the colon above has no ability to empty through it. As I mentioned, it's 1 in 5000 live births. And the ganglion cells here are the uh neuro neuronal cells. Yes, that's the nerves. Imagine they were, they're the, uh, the cell towers uh uh that communicate with each other and pass your signal along as you're trying to use your, uh, your cell phone. The, these, um, ganglion cells can't communicate with each other and therefore cannot induce a peristalsis, which is the movement. The motion of the colon, so the colon sits very still, does not relax and contract, and stays therefore obstructed. It's a, it's a sad thing. It is like somebody is constipated forever. Yes, that's right, the one. Very important difference between a patient with Hirschmann's disease and a patient who is constipated is because of the lining of the bowel that's not normal, the immune lining of the bowel is not normal, the bacteria that stay in the colon can migrate out and get into the bloodstream. And create a very serious condition called enterocolitis, which is life threatening. So it's not just the stasis of the stool and the failure to move the stool through, it's also the fact that the bacteria now have a way of getting into the bloodstream and making the baby very sick, and you have to intervene and stop this obstructive process. And, and does this threaten their life if it is not fixed? Yes, 100%. Uh, and, uh, sadly, if there, there are a few patients who die each year from enterocolitis, but it's, if it's recognized, it's pretty straightforward to how to intervene. Um, and that's, uh, uh, really, really important, and we'll talk about that of, uh, it's not, it's really a medical treatment on to intervene. You don't necessarily need surgery to prevent enterocolitis. You just need a good nursing care and know how to do a proper irrigation to get the stool to flow and overcome this obstructed area. Got it. Here are some pictures of two babies, uh, the one on the right looks quite ill, um, very poorly perfused. They both look very massively distended in the abdomen. So the newborn has no meconian passage in the 1st 48 hours of life, can develop enterocolitis, as I mentioned, and sometimes a little more subtly, they stool a little bit, but not enough, and then they fail to thrive, and the diagnosis is made over the next several months. In older children, um, it can be more of a chronic condition, more of a severe constipation with significant distention, and it can also impact their growth. That's about 5% of the patients, um, present after 1 year of life, the vast majority present as babies. My, my friend whose son had this problem, he used to say that he complains of one, there is distention, and secondly, he complains of severe pain. Is that a characteristic as well? Yes, I think the, uh, the failure to pass the stool and the dilation of the bowel filled with uh liquid and gas is, is very uncomfortable and causes crampy pain. The, the healthy part of the bowel is trying very hard to empty. But downstream, it's unable to get through the obstruction, and that's where the crampy pain comes from. Got it. So this, this part that is healthy, it is trying to push, while the remaining part is really not working very correctly. That's, that's, that's quite correct, yes. Got it. I think it's very important to recognize that um there are some genetic connections to Hirschprung's disease. Uh, here's a list of some of the syndromes, uh, Down syndrome, for example, listed first, there's a rare condition where um the central breathing is, uh, negatively impacted, called central hypoventilation syndrome, multiple endocrine neoplasia, Moet Wilson, Wardenberg syndrome, all of these are associated with Hirschrung's disease. Rarely, there are many patients with Hirschprung's disease have absolutely no connection to a genetic disorder, but it's important to look a baby over that you think might have Hirschsprung's disease and make sure that there aren't other, other issues related to a potential, uh, chromosomal problem. Got it. So imagine you have a newborn who um is in the neonatal intensive care unit or in the well baby nursery that gets sent over to the neonatal unit because they have not passed stool. It's not obviously Hirschprung's disease. This is a list of all the things that it, this could be that cause abdominal distention. Some are surgical and some are medical. You see here, you could have a. Uh, obstruction in the small bowel causing massive distention, obviously you could have an abnormality in the anal opening. I mentioned that at the very beginning of one of the things that we do is take care of patients without normally formed anal opening in a erectal malformation. A meconium plug can occur where there's a very thick piece of stool at the bottom, uh, that hasn't passed. 10% of those patients actually have Hirschprung's disease as the underlying cause, and 90%, they just pass the plug, usually with the help of a contrast study, and they get all better. Meconium ileus, um, associated with cystic fibrosis, could present with abdominal distention of this type. A blockage, a congenital blockage of the colon called colonic atresia, all these are surgical. Then you may have a baby who is um allergic to the milk protein, um, and that mimics Hirschprung's very much so. Interestingly, if you biopsy looking for Hirschprung's disease, you'll find ganglion cells, but you'll find lots of eosinophils. Eosinophils are a cell very much associated with an allergic response, so this is a mimicker in the newborn period of Hirschprung's disease. Um, if the mother received magnesium sulfate to slow down her labor, that can cause a, a paralytic ileus, a slowing down of the colon function when the baby ultimately gets born and look a lot like Hirschprung's disease, a rare condition of small left colon, where the left colon is very narrow, tends to occur in diabetic mothers. And opiates, um, that can pass to the baby from the mother can make the colon very sluggish and look a lot like an like a colonic obstruction. And hypothyroidism, so a low level of thyroid can make the colon not function well, so all of these things need to be assessed for. Obviously, today we're talking about Hirschprung's disease, so this particular x-ray shows an impressive abdominal distention. And ultimately this baby turned out to have Hirschsprung's disease, and what you can see most dramatically is the very dilated colon. That's right. This is, uh, am I correct on, uh, putting the mouth scar on the rectum and sigmoid there, um, and then the rest of the colon is also dilated. Got it. So let's, um, let's, let's say the patient has in fact a suspicion of Hirschsprung's disease. We need to make the diagnosis, and, uh, this is what that, uh, the next slide is what makes that, um, diagnosis. There is a, a special suction, uh, gun, uh, invented by Doctor Helen Nobleblett from Melbourne, Australia, where a, a very tiny, um, uh, metal, uh, tube is inserted into the anus. And you see on the left side, the anatomy of where you want to biopsy just above the anal canal, and you get a piece of tissue of the rectum that's pulled into the gun, and you look under the microscope to see whether you can find those ganglion cells we were talking about before. And if there are no ganglion cells present, once that piece of tissue is analyzed, then you confirm in fact that the diagnosis is Hirsprung's disease, but in addition, you can't only have the absence of ganglion cells, you also need confirmation that the nerves associated with those ganglion cells are thickened, hypertrophic is the term, so you want both those entities and a pathology diagnosis to confirm that this is Hirschprung's disease. Got it. So this is actually, you take a piece of tissue from the anal canal area to confirm if the neuronal cells and ganglion cells are there or not. That's right. So you take a little piece of tissue, you have your pathologist look under the microscope, and they're looking for those ganglion cells, and they're looking for those nerves. And if there are no ganglion cells identified and the nerve trunks are thickened. Uh, greater than 40 microns is abnormal, then that makes the diagnosis of Hirschsprung's disease in the clinical setting that you ended up biopsying for. So a baby that's not passing meconium, or one that has an X-ray or a contrast study that is suspicious. Got it. Thank you very much. So, um, if the suction biopsy, if you can go back just one, if the suction biopsy, um, gives you the answer, great. If the suction biopsy specimen is too small, or if you're trying to make this diagnosis in someone older than the age of one, you need to do a more formal biopsy in the operating room, you take a bigger piece of tissue. So there's a difference between whether you take a suction rectal biopsy. With the patient in their normal uh uh bed in the neonatal unit, or they need to go to the operating room for a bigger piece of tissue, uh, called a full thickness biopsy. In both cases, you're looking for the anatomy that I just described in the next slide. Um, there's different ways of processing the rectal biopsy. Um, when you're doing the actual operation, we use something called frozen section. Because what we need to know at that time actually is not whether the patient has Hirschsprung's disease, we've already confirmed that. Now we need to know, have we reached the healthy bowel, so we can immediately get an answer from the pathologist that yes, that piece of bowel is healthy and there are ganglion cells, and then we know that that's the segment of bowel that can be connected to the anus with the intervening segment that was causing the problem removed. A permanent section takes about 2 or 3 days to analyze with various um slide preparations, and that's the one you really need to be absolutely certain that it is Hirschprung's disease. So I like to say that you cannot um diagnose Hirschprung's with a frozen section, but you. Can rule it out, i.e., if ganglion cells are present, but to prove that it is in fact the condition of Hirschprung's disease, you need several days, and you need to evaluate 100 slices of the sample that you took, so you don't go into the operating room unless you know for certain that the patient has Hirschsprung's disease. Got it. And this, uh, the, those 100 slices or more, are they taken in one, procedure during one procedure, or they are taken? Well, they're, they're, they're cut from that sample that I described. So you take that and then you cut it into 100 different s with a micrometer. Uh, you're, you're taking very tiny slices, um. Um, with a special device that makes very, very thin slices, and you look at 100 of them, and if you don't find a ganglion cell in all 100 slices, you confirm it's not, uh, you confirm that there are no ganglion cells there, and it could be Hirschprung's disease then. Only with that much analysis can you prove it. Got it. So we have a fun question from Rema, if you don't mind. The question is, is the good doctor Canadian? Love how he says out. No, I, if I was Canadian, I would say oat. Um, my, my out is a little bit more American, but no, I'm not uh not Canadian, but very fond of that country. Yeah. Agreed. I have, I have relatives in Montreal if that counts as partial Canadian. Yeah, my brother is a Canadian as well. So, um, we talked a little bit about this. This is a little bit more of a sophisticated slide, but, um, the images show, um, that you're looking for both the, um, absence of ganglion cells in the various layers of the bowel and the presence of thickened nerves that are greater than 40 microns. Got it. So how do we see that which one is a ganglion or which one is a nerve? Well, there you, you see, um, I think the best probably one is an E. There's that thing that has the arrow on is a nice looking ganglion cell, uh, uh, to the right there, there's a very nice purple circular ganglion cell in the E on the left. And then, um, there are some hypertrophic nerves in the, in the slide on the top, um, the, there's a, there's a cluster there of, uh, there's a little N, you can see the N in the very middle of the slide that looks like a thickened nerve. You need very sophisticated pathology to. Uh, to determine this, uh, pediatric pathologists are very specialized in Hirschprung's disease, and we're very dependent on them because without a good pathologic diagnosis, we really can't do good surgery. Got it. Thank you very much. And there are some other ways of staining, um, not that vital for this particular talk, just for completion's sake, there's cal retinin staining and, um, acetylcholinesterase staining, um, all of these can be done and, and all put together can make the determination of whether the bowel is normal or whether you're dealing with a case of Hirschsprung's disease. Got it. So, um, this is a, um, a nice image which you see a lot of the colon is narrow here. Um, it's not very obvious that any colon is large versus small, so this is a, um, an image that really speaks to that maybe the entire colon is involved with Hirschberg's disease, because, um, as I showed you in that original X-ray, the narrow part was at the bottom and the dilated part was at the top. Here, everything looks narrow, so maybe the entire colon. Um, is contracted and, uh, and does not have ganglion cells, so the vast, vast majority of patients, the problem is in the lower part of the colon, the left colon or below, and in about 15%, it's higher than what I would call the splenic flexure, which you can see is in the top left corner of that X-ray, but this particular image, the entire colon is narrow, this probably is a case of what I would say is total colonic Hirschprung's disease. The entire colon is involved. Got it. And this is a more typical image of a classic type of Hirschrun's disease, where you can see the narrow portion, all that squiggly stuff, and then it merges into a more dilated portion in the left colon. So all of the large part is healthy, normal, trying hard to push through the contracted part. So this con the transition zone is the word we use for where the. Good colon merges to the bad colon. The transition zone here is the very distal left colon, where your cursor is right now, and the rectum and sigmoid are all the squiggly part. That's abnormal, no ganglion cells present. That's the part that needs to be removed. And it's important to remember that this contrast study is accurate, but only about 90% of the time. So when we do the actual surgery, we need to confirm under the microscope where the healthy bowel begins. Got it. So I have a question here. This bigger size of the colon, is that because of, because it is filled with the material, or it is filled with the material plus it has been trying for a longer time and so it is, yes, it's dilated because it's trying hard to empty and it's not emptying successfully. So here it's filled with the contrast material and it is that size. And it will shrink down very nicely to more normal size, as soon as it's given the opportunity to successfully empty out the anus, once the blockade has been removed, and the blockade here is all the a ganglionic, the non-ganglionated segment, which is the squiggly part you can see. Got it. So, let's say we've confirmed the diagnosis, and now we need to um operate, and the baby needs to be doing well, healthy, uh, not, no longer distended. So it's during this period of time in the neonatal unit where the patient is undergoing irrigations, um, we we pass a soft catheter, um, up the anus and irrigate to take the, uh, fluid and gas out. And decompress the larger colon because we have overcome the obstruction by replacing the lumen with essentially an irrigating irrigating tube and therefore now the colon can successfully empty, the baby feels better, they stool, however, with help. And now they're ready for their pull through, and we call it a pull through because the healthy segment is pulled through to the anus and remove and we remove the abnormal segment. So if I go back for a second, if you don't mind, if I go back here, this part that is squiggly small part, and this part is all removed. Yes, I think you have more pictures later on, and this part is then connected. That's right, that part is brought to the anus. And so the pull-through term means to pull this part and bring it forward. That's right. Pulled through. Got it, and I have a question. Our team, um, our team shirts say you won't believe what our team can pull through. Interesting. And thank you very much that this is a very, very important, uh, work that you do. I have a question here as well. Um, the question was. By here. So, Ramnick says, would differential diagnosis include iatrogenic nerve damage, although not for newborn, does it present later in life? Yes, I, I don't, I don't know what, uh, uh, what the cause of an iatrogenic nerve damage that you're referring to. Uh, so, no, in these newborns, uh, I, I went through the differential diagnosis, um, but if the ganglion cells are absent, then they are congenitally absent. They never formed. Got it. So the chances of getting them damaged after they had been formed in a healthy baby, is that also possible? No, that, that I've never, I've never actually heard of, no. Got it. And just one more question, and you may have actually the answer to this later on. Doug Gross says, pull through, is this done through a scope? Yes, so, uh, one of the techniques that is typically used is a telescope called a laparoscope. You look inside the abdomen, and then with with instruments as thin as a pencil or a pen, you manipulate the bowel and dissect it to the point where it can be pulled through. And then, of course, you need to do a little work on the anal area. That part is called the transanal dissection. So through the anus, you core out the bottom part and then pull through the entire colon. And, and some of the surgical options are to create a stoma, a stoma bag, and do the pull through in the future. The other option is to do the pull through straight away right there in the newborn period, and the third option is to irrigate and keep irrigating and let the patient go home and be on irrigations at home and come back 2 or 3 months later for their pull through. All three of these are very, very reasonable options. My personal preference. Is to do a primary pull through if the baby is well, um, and, uh, and the baby goes home essentially with the Hirschrun's disease, uh, eliminated from their, from their body. Got it. So, ostomy would be that there is a bag attached to the healthy part. That's right. So that, and it would probably be outside of the body. That's right, and the, so the piece of colon sits on the outside of the abdomen. You can actually see it, it looks like a red circle, and then, uh, the stool empties into a bag, and then at the time of the pull through, that piece of bowel is brought down to the anus and the distal segment, the unhealthy part is removed. Got it. And, and there is, uh, probably you have that discussion further, that what will be the indication to not do the primary pull through and go for the other options? Well, the, the issue really is, um, how well is the baby doing. If the baby is, is unwell and not responding to irrigations, or has developed enterocolitis, then your best option is to do a, a bag, a stenostomy, let the baby recover, and then do your pull through. I see. So it depends upon the baby's situation. And, and this uh uh picture shows that pull through the left side image you've already seen, the right side image is now with the abnormal segment removed, and the healthy segment brought through. Um, and I think it's pretty clear now what we mean by a pull through that healthy segment is brought down and connected to the anus. Got anal canal and the sphincters are preserved, so the patient should maintain it, uh, their bowel control. Got it. And because it is done in younger children, I'm just assuming and making up a question that came to my mind, because it is done in, in the younger children, do they then spend their life as normal as others? Yes, 100%. You really only need about 10% of your colon to function completely normally and have one bowel movement per day. Most of these patients only lose about 15 to 20% of their colon because that's where the abnormal segment is. So, yes, and the colon will continue to grow. So, yes, they can have a completely normal, uh, uh, pooping life, uh, stooling once per day. When you have a patient who has to lose their entire colon, um, and bring the small bowel to the anus, those patients tend to have more frequent stools, somewhere between 2 and 6 a day. Um, uh, but they can all maintain bowel control, provided that the surgeon successfully preserves the anal canal and the sphincters during the operation. Got it. And just for, for our interest, I think you have done 10,000 or more surgeries. I have not all of Hirschprung's, but um, yes, I've been basically doing uh 500+ operations for the last 20 or 25 years, so that's correct. So we mentioned this already, um, there are certain circumstances where babies are, do not respond successfully to irrigations, and they may need a diversion, the stoma bag, so we actually went over this already. Um, and this is, uh, an image of what an abdomen of an X-ray, abdominal X-ray might look like, if a patient is not responding to irrigations, and you can see the massive dilatation of the colon. This should be decompressing with your irrigations, but sometimes the irrigation tube does not reach high enough and does not get into the normal bowel, and therefore, you can't successfully decompress this bowel, and this is a patient who probably would then benefit from a diversion with a stoma. Am I correct in outlining that this whole thing is enlarged colon? That's all colon. That's right. And interestingly, if you look carefully, there's not a lot of air in the distal segment towards the pelvis, and that means that that's probably the bowel that's decompressed and, and narrow and fluid filled, so you don't see air in it. Am I correct in, uh, circling this part, so you're, you're pointing out here? Yes, right there, there's nothing. I don't see any air at all, which means that's the a ganglionic, the segment without the ganglion cells. Got it. Thank you very much. So this is just a um picture more for the surgeons to understand the anatomy of where you take your biopsy, and the different levels of the bowel, uh, from the epithelium in the inner layer, the mucosa, and the adventitia on the outer layer, and you want to make sure there are ganglion cells throughout, and nerves that are normal throughout. And you select your piece of bowel that you're doing your pull through and for the questioner who asked about uh telescope and all that, those are what the instruments look like under laparoscopy. You can see these tiny fine instruments are actually taking a biopsy, but no incision has been made on this child's abdomen. Got it. So, a couple of questions. There is one question from Rema. What are ganglion cells? So ganglion cells are basically um a nerve, uh, nerves, um, well, I, I like to I like to think of them as the uh. As the uh node, the um, the, the tower for your cell, uh cell service, uh, each tower collects all these different signals coming from all over, all in one tower, and then that jumps to the next tower, so the tower is. Basically the ganglion cell, it's a compilation of where all the nerves funnel all their messages and if you don't have ganglion cells then you can't provide the signal for the bowel to relax and that's where you get your obstruction from because the bowel is, is perpetually contracted. Can I quickly, if you don't mind, uh, so the cool beans are aware of my, my Fascination with drawing. So Rima, let's say this is the. Colon, and my apologies, it's not supposed to be anatomically correct. So let's say this is the tissue, and in this tissue here, we have nerves. And, and our GIT is actually very rich in nerves because the whole GIT needs to be coordinated. When the GIT is working, so we cannot afford that any one part of GIT is working out of sync with the rest of the part. So what happens is, and then there is control coming from, you know, the sympathetic parasympathetic system. There is control coming from within the segments. So what happens is the nerves collect in one area and they relay their signals, and then from there those signals are picked up and as Mark was saying they are then further processed and relayed, and they would then go to various muscles or other places and convert convey those messages. So this relaying center, as Mark is saying, a, a tower, cell tower, this is a ganglion. So ganglion is really when we used to do the definition of nervous system tissue. Gray matter outside of the brain, or the processing neurons, a cluster of neurons, bodies, or the processing centers outside of the brain is called a ganglion. I have one more question, Mark, if you don't mind. That question is, From Bambi. She said it seems painful. Let me see if I can find her question. It's scrolled up, but she said it's, it looks painful. So is it, how is the baby's experience during this whole process? Well, usually if you have successful um irrigations, then you are solving the distention and the baby is quite comfortable. um, and that's a signal to you that it's time to go to the operating room, uh, and, uh, fix this baby and remove the Hirschprung segment. Got it. Thank you very much. All right, so we talked through this. This is the treatment of abdominal distension. I think we have some, Images of what an irrigation looks like. This is a really crazy picture of a tiny little baby, and this is stool under pressure. Um, and here you can see that this baby had all of this stool built up inside and once we put the tube in and sort of uh relieve the obstruction. You learn with experience that you make sure to examine these patients by standing on the side, not at the feet, otherwise you'll get sprayed and you see this, how far away the stool reached. Um, um, it shot all the way across the room because this, uh, uh, stool was under pressure. Uh, because it was, uh, trying to empty against an obstruction, and you need to break that cycle. And is the baby in pain during all this time? I think they are they relieved uncomfortable. They're uncomfortable when they're distended, and they feel really relieved once you successfully irrigate them out. Got it. And that's what the irrigation looks like in a, in a mannequin, um, you pass a tube in the anal canal and get the fluid to flow, um, and, um, thereby essentially overcome the obstruction by putting a tube in the channel. Got it. So in, in this diagram, the way I would uh visualize it is imagine you're sitting in traffic and an ambulance comes along and the traffic parts to let the ambulance go through, and now you are no longer obstructed, at least for that ambulance, um, all the way through that traffic. That's what that tube is doing. Got it. And there is this open end over here, so does it actually help relieve as well, or it is just, yes, you put the fluid in and then you take that syringe off and you let all the liquid stool that's built up exit the tube into that uh metal bin there. Got it. And you have written here, irrigation is not enema or, or enema. That's right. That's right. The irrigation is a process whereby you're irrigating and gently getting the stool out and enema, you just squirt it in and walk away. That's certainly not going to work in a patient with Hirschrug's disease. So this technique is vitally important and it's a good reminder that these patients really good, need good surgeons, but also need very good nursing care. Got it. There's a question from Paul Borg. Is this an inheritable disease? Yes, we, we mentioned that a little bit earlier when I talked about some of the uh syndromes that are associated with Hirschbung's. Most are not heritable. Um, however, um, some are, and, um, you know, a family asks me if they have a baby with Hirschprung's disease, what's the risk of another baby having Hirschsprung's disease in their family? It's about 1 in 200, so certainly that is a small number, but it's significantly higher than the the risk of the general population, which was 1 in 5000. So some cases are heritable, most cases are not. Got it. Thank you very much. So, here is a uh another picture of very significant distention and the treatment of this um distention now, which has now become, I mentioned this earlier, and uh Hirschprung's associated enterocolitis, HAEC and the treatment is to do irrigations. And to give an antibiotic, uh, typically the one that's used is metronidazole, which is for, um, anaerobic bacteria in particular. Hydrate the baby with intravenous hydration, make sure they stay well hydrated, and then on rare occasion, this can occur even in a baby who has a stoma, so we need to be suspicious of why they would be developing enterocolitis in the stoma, which in theory should be an easy flowing system, maybe there's a stricture. Um, and then maybe the bowel that's downstream is getting into trouble. So again, these are a little bit more, uh, complicated scenarios, but suffice it to say it's, uh, it's, it's the obligation of the clinician to make sure first to try to prevent Hirschsprung's associated enterocolitis, but if it is to develop to properly treat it because this is where, um, you can, you can lose a baby from enterocolitis. That's why they need the surgery to prevent this problem, uh, from happening. Got it. So, um, after we've done the surgery and we've done the pull through, um, there are some early, uh, surgical consider post-surgical considerations, um, we're gonna have another session, I anticipate on the longer term considerations, the longer term outcomes such as fecal continence and constipation, etc. but in the very early period. There are a couple things we have to worry about, and the next slide I think shows that. Um, there are some patients who, even after a very successful surgery, need improvement in their emptying because their sphincters don't relax well, and it's another part of the Hirschrung's condition, and this is a view of the anal canal and a very funny picture of uh two dogs, one before and after they've received their Botox. Um, but, uh, occasionally you have patients who, um, have had successful surgery and their sphincters fail to relax until the baby learns how to relax them at the appropriate time. And even after successful surgery, you are in danger of getting enterocolitis if you don't have good flow, and the sphincters can slow down the flow enough that actually you can develop Hirschprung's associated enterocolitis even after surgery. Well, the treatment once again is irrigations, but then we know that if we give Botox to temporarily knock out the sphincters and the Botox wears off over the next 3 months, the baby can learn to push on their abdomen, use some of their abdominal wall muscles, and push through and overcome these non-relaxing sphincters. Very interesting. So the baby originally had not learned to empty themselves because they never actually had a chance to do it. Yes, I think that's part of it. And also they, there is an abnormality of the sphincters that is part of the, of the condition. Um, the internal sphincter does not relax normally in Hirschrun's disease. When you have fullness in your rectum. Your internal sphincter is supposed to relax, and your external sphincter, the one you have control of, is then supposed to contract as you find a bathroom. In Hirschrung's disease, interestingly, the internal sphincter tightens at the wrong time and can hold the stool in. At an inappropriate time and actually can make the baby distended even after successful surgery, so we're all uh surgeons aware of this and know that on occasion we have to intervene and the intervention that's successful is either an irrigation or to administer Botox into the anal canal as you see depicted in this picture. Got it. Thank you very much. So that um really concludes the, you know, the newborn assessment, the making of the diagnosis, the ruling out the other possible conditions, i.e. the differential diagnosis. The surgery that's required to solve this problem and then some of the early issues that may need to be intervened on even after successful surgery and in another session, we're gonna talk about the recovery and what to expect over the next few years. The good news is the vast majority of these patients recover very uneventfully. They stool normally and when they get to the age a little bit older, age 3 or 4, they successfully potty train. Very successful, uh, clinical result. Thank you very much for this. I have a couple of questions and, uh, cool beans, if you have any questions, please let us know. One question that has been in my mind, and I am a little disconnected from the surgical area, so, uh, please pardon my ignorance. So here you and your colleagues, you are, you are experts and you're helping babies. Worldwide. Do every baby get a similar treatment and management, or do they end up dying because the facilities or doctors are not aware of how to manage it? Um, there's no question that this is a source of significant both morbidity and mortality in the developing world, um, if it's unrecognized, um, and, um, however, if it is recognized in the vast majority of locations, uh, the surgeons know what to do. They know how to irrigate, they know how to intervene, they know how to make a stoma, they know how to do a pull through as we described, um, you know, I'm, I'm really working very hard on. On getting this expertise out there, we have a, um, a team uh called Colorectal Team Overseas, uh, from, uh, represents a number of countries that travel throughout the world and uh particularly focused on going to the developing world and training surgeons and nurses on the skills to take care of patients like this. But if the diagnosis is successfully made, the interventions are very reproducible, uh, and the babies can do extremely well. Got it. Thank you very much for this. Um, a question from Rema, it must take special skills to work in such wee ones. Hand-eye coordination must be the skill this dog has. So, what are the uh differences from adult surgeries and especially in this area? Well, yes, well, everything's, everything is much smaller, um, and, um, much more intricate detail, uh, very fine movements, uh, more elegant instruments, uh, and it takes a special training. I had to do, uh, uh, 5 years of my general surgery training for on adults and then did 3 additional years, um, to learn how to take care of children. Um, and, uh, that was about 25 years ago that I finished all of that, um, and, uh, so yes, but, uh, pediatric surgeons take care of the smallest of babies. I've, um, operated on, uh, a baby the size of my hand. So a premature baby weighing, you know, 800 g, uh, is quite small, but they sometimes need surgery. Hirschprung's patients are mostly full term, so that's about 4 kg, um, about, uh, 7 to 8 pounds is when we do the operation. So, um, uh, that's may seem small to an adult surgeon, but for a pediatric surgeon that's a very good size, and the patients I like operating on kids because they're much better patients and they recover much quicker than adults. Got it. Thank you very much. We used to say that when I was, uh, doing my work in the hospital, that the pediatrics, so doctors from the pediatrics would say, uh, we can do more successful CPRs that our patients recover better and faster and, and they have better outcomes. So it is good to know. There's a question from John Doe. What is the distance from the internal to the external sphincter, infant through adult? So this is an anatomy quiz for you. Yeah, they're very, they're very um much connected. The internal sphincter is the very end of the bowel where the anal canal forms. The external sphincter surrounds the anus. The internal sphincter we have no control of. It's uh very important because we therefore don't have to think about our anus uh during the day and no stool will pass because it remains contracted. When it's given the signal that there is stool in the rectum, the internal sphincter relaxes. And as soon as it does, the external sphincter, which is a ring of sphincter right outside of it, so right next to it, um, is going to squeeze until you then find yourself a bathroom. So the, I would say that the, they're intimately connected to each other, one surrounds the other in infants and adults, but the zone of how long the sphincter is obviously is longer than in an in an adult, than in a child. Got it. Thank you very much. Um, Karen says, how do you explain a total colonic Hirschsprung diagnosis in a child who eventually passed his meconium in the first week of life? Yeah, it, it, that's a very interesting question. Total colonic Hirschprung's is somewhat mysterious sometimes. I've met patients that pass stool somewhat successfully, not that efficiently, but somewhat successful for many, many months. Who turned out to be have total colonic Hirschprung's disease. I don't think it's something we truly understand, but there are definitely babies that you are, uh, alluding to that did pass meconium, but they limp along and they don't really thrive, and they don't efficiently stool. Some stool does come out, which is why they don't get acutely ill, um, and the diagnosis is often delayed when it's a total colonic case, that's true. Got it. Thank you very much. Couple of more questions. I know that you're on the East Coast and I know that you just came back from the hospital before this talk, so it is late, plus you're tired. We have lots of questions. So a couple of more questions. Bambi says. Why do pediatrics recover quicker with most things compared to adults? Is it related to their collagen and better wound healing, etc. Yeah, that's a good question. I don't know the answer. I think perhaps it has mostly to do with it. They don't have comorbidities. Um, there's usually an isolated problem. They have good hearts, good lungs, good kidneys, good blood flow, all the things that you need to have a good surgical recovery, whereas in an adult you might not have those things and you're still trying to recover from an operation. Um, it's just something we know that children, uh, bounce back much quicker, um, than, than adults, and it's probably related to the other things that are going on with that adult that kids tend not to have as frequently. Got it. Uh, the Colombian coffee bean says, can maternal drug use cause Hirsh brown? No, that's, uh, that's never, that, that's never been reported. I've never I've never heard of that. One last question. I said two, but this is the 3rd 1. Was this surgery developed on cats, dogs, or other possibly animals? Um, I don't believe so. I'd have to look up the history of, um, Doctor Swenson was the one who came up with the idea to on how to fix this surgically. I will tell you an interesting vignette. I was once invited. To speak to the American College of Veterinary Surgeons, I received an email once from a, um, back to the Canadian comment. I received an email once from a Canadian veterinary surgeon that said, um, I've been reading some of your papers and it seems like you have very good results in maintaining continence in children. We have um some uh dogs that need surgery on their rectum. Could you help us? And I had a fascinating. Uh, visit, um, with to their Congress and talk to them about anal surgery in babies and they, uh, and they took some of the techniques that I had described and started to apply them to, um, um, this similar, some similar surgery in dogs. So it was a proud moment and as a dog lover myself, I, I felt I helped a bunch of dogs with that with that trip. But your question was whether the um surgery was developed in dogs. I will tell you, I do know that in imperfored anus, the other condition that we mentioned, um yes, those patients did undergo, um um there were surgeries on uh beagles, uh, as a first step and showed very good results before they were applied uh to humans, but I don't know the Hirschprung's, uh, history. I can check that for you. Got it. So thank you very much. Uh, so once again, Mark, thank you for your time. Thank you for giving us time when you just came back from the hospital. I've been thinking about it since then. And Cool beans, thank you very much for joining us with the session as well. We'll do further session to continue to, uh, explore more of this area. So Mark, thank you very much, my great pleasure, and I really think you're doing quite a wonderful service to, uh. To educate the world on some of these difficult topics and um I'm really fascinated by the level of, level of excitement and interest, and I appreciate you uh letting me uh become a cool being myself. Thank you very much. So, by the next session, we'll figure out a name for you. Perfect. Very good. Great. So thank you very much, everyone, and I will see you next time. Bye-bye.