Chapters Transcript Video Better Lives for Babies With Anorectal Malformations: The Value of Early Identification I'm going to introduce our speaker. Dr Sarah Cairo is actually brand new to us and she um is uh did training and minimally invasive procedures for newborns and Children. Um She's interested in improving access to care for pediatric patients with complex colorectal disease and creating teams to meet their needs. So she's also part of our UCSF Center for Health equity and Surgery and anesthesia and she has a lot of stuff going on and I'm so excited to have her doing with this talk for us. So I'm gonna stop sharing my screen and let her take over. Hi Dr Cairo. Hi, thank you so much Tabatha, let me Sure. Um So as you know from the fire and the introduction um today we're gonna talk about in erectile malformations. Um There's obviously a lot to digest and that can be discussed in this topic. Um But what I'm hoping that we'll be able to focus on are some of the things that as a community pediatrician, as a primary care provider um are really important for you to be aware of or be thinking of. Um So we'll go through some of the nitty gritty and then hopefully we have some room for discussion at the end. I have no disclosures. Um And so before we get into the weeds of embryology and the management and even the outcomes. I wanted to go over to scenarios that are really why I think that this is a critical topic for discussion with pediatricians. Um And obviously if we were in a room, I'd ask you to raise your hands and tell me who does in patient care and who's responsible for newborn exams. But since we don't I'm going to assume that this is outpatient providers. Um So for those of you who are outpatient providers you probably do some new patient evaluations shortly after deliver your discharge. And in either case these patients that I'm going to describe our patients that you may see in clinic um or who we've been called to the emergency room to see more frequently than I would like to admit. So the first patient is a three week old female who we saw in the emergency department for abdominal distention. And the patient was born full term at an outside facility but vaginal delivery was discharged home from the hospital the day of life. Tomb was reportedly stooling normally at that time. Um The parents however, noticed that the patient was seeming uncomfortable maybe doing some straining and that the stools have been getting thinner. They described them as sort of pencil or like a piece of spaghetti. Um At one point they noticed that the baby had a low grade temperature. So they called the pediatrician on call who recommended that they come into the emergency department. Um Their initial concern based on the parents description was for U. T. I. But it's a three week old with some changes in stooling and fever. So they suggested they come into the murder, the emergency room. The patient was overall well appearing and the emergency room doctors got an abdominal x ray um which is really non specific bowel gas pattern. Maybe a little bit of constipation. Um I'm gonna show another X ray for a patient with the same story. Um That shows a bit more bowel gas distention and also some constipation. So the emergency room doctors ordered a suppository for the patient thinking that this was mostly related to constipation. And the nurse called back to say actually I'm not sure where to put this repository. This patient doesn't seem to have a normal anus. Um So the surgery team was then consulted for concerns for an erectile malformation. Um This is not the actual patient. Say this is sort of an example of what we may find in this situation. So there's no clear anal opening. And when we examine this patient from that lens were able to identify a rectal vestibular. Um So this sort of patient will be admitted to the hospital to the surgical service and we'll take them to the operating room to to perform an exam under anesthesia and confirm the location of that fistula in the absence of a normal opening. Um And unfortunately being three weeks old and having that sort of distended colon, this patient would get a diverting colostomy. The second case um is one that I've actually had here in San Francisco. So this is a three day old male who was referred from their pediatrician with concerns for anal rectal malformation. Um so this baby was born at 38 weeks I. Vaginal delivery pregnancy was notable for some gestational hypertension and maybe some pre eclampsia. Um No mom has a history of congenital heart disease and maybe an asd repair um as a one year old. Um But this patient, the baby had had normal prenatal work up in care, a fetal echo that was described as normal. Um The patients spent one night in the NiCU for hypoglycemia was discharged the next day pediatrician saw the patient on day of life three and was concerned that the anus didn't appear normal and the baby had been stooling. So it hadn't really been picked up on by the NiCU or by the newborn nursery. Um But they requested that the patient comes to the emergency room. Parents said that the baby had been feeding well, um had been stooling a few times a day and they really had no concerns at that point. The emergency room. The patient had a soft non distended abdomen. Um but on perennial evaluation had really a pinpoint fistula just posterior to the scrotum. The patient was taken to the operating room the following day and was able to have a primary anal classy because at this point they have a fistula that was easily accessible and um we're only day of life three. So I didn't have a great deal of bowel distention and worried about she was able to be discharged on day one and has been seen in follow up and is starting some anal dilation and overall doing well. So how does this happen? There are obviously several layers to how this can happen and how these patients can be missed. Um And part of the problem is that when you have a patient who's doing this is what you may see in the delivering room or in the newborn nursery um to the new parent. Again this may be what they see and depending on the parents education familiarity they may or may not know what they're looking for. Um And what may be abnormal the outpatient provider. Again if the baby schooling in your clinic um some people may not go so far as to do a thorough evaluation and sometimes these malformations are quite subtle and so we acknowledge that they can be hard to pick up on. Um And so part of this talk is to um sort of give you the power to make you feel able to send us any patients that you're worried about or aren't sure about. Um And this is what we're talking about this what you can see here these red things are supposed to be the sphincter muscles. And in some of these patients with the fistula you may see a separate opening as an anus and be unsure if it's in the right spot or if it's big enough it's what it's supposed to be. Again that's why these can be sort of hard to pick up on initially and why does it matter if you notice it in the delivery room or in the newborn nursery or if it gets picked up on three weeks later when the patient isn't fooling. Right? And as you saw in those two examples, it really does make a difference in terms of what kind of surgery we can offer a patient and for a family with a newborn, a colostomy versus a primary repair is a big deal and it can have long term consequences for them. Um So it really is relevant in terms of the timing of repair. So with those cases in mind, our objectives for today will be to define the anatomy and some common victorian for in erectile malformations. To review some of the complications following in erectile plastic. And hopefully we'll spend a fair bit of time identifying some of the short and long term outcomes and opportunities for improvement and engagement between a multidisciplinary team. So starting at the beginning with some epidemiology um indirectly malformations affect one in every 4 to 5000 live first, there is a slight male predominance um about 1.2 to 1 and we'll go over some differences in terms of the anatomy and the most common presentation for males and females and males. It's the record. Urethral fistula is the most common in females. The record of vestibular fistula, there are some genetics and some risk factors that we think are related to development of an an erect a malformation. So for example, in a family who has one child with one um there's a 1% risk of the second child will so significantly higher than in the general population. Um there are some autism dominant inheritance syndromes such as Karina's triad. Um that can have an increased risk of these anal rectal malformations. And then in things like trisomy 21, so a little over 1% of patients with trisomy 21 are found to have an erectile malformations again much higher than the general population. Um And compared to the majority of patients with anal rectal malformations, Patients are much more likely to have an arum without official to um that being said, only 40% of patients with an Arab without official, it will have trashed only 21. So um not equivalent but certainly something to think about when you see those malformations and there are some environmental exposures um that we think are related to arum some maternal diabetes IVF and solidified exposure. So how do we define an interrupt a malformation? Um You'll hear the words used interchangeably, but the truth is they're not the same. So you'll hear us say in prefer anus um which is not to say that there is no anus. It's just that it's not normal. So incorrecta malformation is really any absence of a normal anal opening. So that could be that it's non existent but there's no opening at all. It's usually that the the G. I. Tract or distal rectum empties anterior early into either the perineum, the vagina or the urinary system. And that's the fistula that we'll talk about where you can have rectal atresia. So our understanding of this is a little bit interesting. So our understanding of the path a theology behind the development of interrupting malformation that their most extreme, or cloacas are extra fee um really requires understanding the embryo logic origins of all these structures. And I'm not going to spend too much time on this because I think I'll start having palpitations and flashbacks. But there's a long held belief and understanding that the early development of the hind gut, the urinary tract and the reproductive tract coalesced into this common channel called the claw waka. This structure persists until about the seventh week of gestation when the anal rectal septum or the euro general sinus uh shown here is the sort of pink structure. Um I agree it's costly to divide the anterior genital urinary sinus from the posterior rectum. There's then this extra dermal covering this surface called the clerical membrane which eventually ruptures to create the genital urinary sinus or opening anterior early in the posterior anus with a perennial body in between. Um the euro genital sinus then goes on to differentiate into the bladder prostate etcetera. All of our understanding of the embryo logic process in normal development, our understanding of the ideology of these errands is somewhat less clear. So there are multiple theories for the development, including a faulty development of that Euro rectal folder, that anal rectal septum that migrates. Other theories suggest that it's the migration of the rectum towards you know, opening that's problematic. And then there are some newer theories emerging to suggest that it's that ectodermal surface or that clinical membrane um that's supposed to rupture and that that may actually be more important in the development of these malformations than the process of temptation. Um So well it should be fairly simple to classify these lesions. It's a little bit more nuanced and challenging. Um And part of that is because there are obviously differences between male and female. Um but also because of this concept of high versus low malformations. And this distinction is really used by the surgeons to help us understand um how we can approach these operatively and also to help us understand the long term the long term outcomes for the patient. And so the questions that we ask when we class by them or when we evaluate these patients is where is the distal rectum. What is the relationship between the official and the rectum and the urinary track. So where do these these structures join? And and then what's the relationship to the elevator muscles. So not only are their elevator muscles, how well formed are they? Um but depending on where the specialists, it's relative to the muscle groups will help us understand if we can access officials from a perennial approach or if we need to do an inter abdominal procedure as well. So we'll go through some examples and the the details here won't be that important for you. But I do again think it's a little bit helpful to sort of understand the language that we're using. And then when we start talking about what the patients can expect post operatively this will sort of make a little more sense. Um so for a very small percent of intellect and affirmations and males. Um well not they won't have official. And again these patients are more likely to be the trisomy. 21 patients um that's about 5%. And the percent of patients love a perennial specialist. And this image again you can see um what's red here is supposed to represent distinct or complex. And what you can see is that there's an opening but it happens to be outside of that muscle complex. And so while a patient may be discharged with this anatomy from the nursery because they are stooling um this opening will always be too small and the fact that it's not located within a muscle complex means that they will never have normal control of their bowel function if it's left like that. The most common malformations. Again in males are gonna be the urethral fistulas and these can be considered lower high depending on where along the urethra. The fistula is um This distinction between rectal boulder and rectal prostatic um is not only important in terms of understanding surgical planning but does sort of influence the long term outcomes of the patients and their ability to achieve continents. Finally, in this high category, high category will have directo vesicles, fiscal or bladder fistulas um which are often associated with really underdeveloped sphincter muscle complex and really um poor outcomes. In terms of emails. The indirect informations, we again have the relatively uncommon case of anal atresia um Or no fistula. You can see here About 20% of anal rectal malformations and females um will have a perennial or cutaneous fistula so similar to males. It's easy to imagine these patients being discharged home because school is actually passing out their bottom. Um but again, the fact that it's a cutaneous official means it's less likely to dilate or be functional and again, it's not surrounded by normal sphincter muscle complex. So these patients um could never realistically be expected to achieve content. Recto vestibular fistula, on the other hand, is officially located within the posterior aspect of the vestibule. It's not located in the skin or in the true vagina, but really in the back um here. Um And then lastly will be the cloacas. Unfortunately these aren't terribly common, but they are among them are challenging for repair for long term management. Um And in the case of coca, there's a single orifice emptying the bladder, the vagina and the rectum. Um And this is important to distinguish from a true recto vaginal fistula, which is presently rare. Um That needs to be distinguished from between recto vestibular recto vaginal and awake to make sure that the patient really gets the right operative repair and that that you're a genital sinus is recreated. So once we have a suspicion of an indirect um information whether in the clinic or the nursery the work of commences and consists of really two major components. Um The first is around surgical planning and depending on what the bedside exam is like. Um We may need to do an exam under anesthesia and the real goal in that procedure is to stimulate the muscle and identify where this opening official is relative to the sphincter complex. Um And this can help us differentiate between an anal latricia and anal stenosis or just a small anus from a true perennial fistula. Next we may do some imaging studies. So you may hear us talk about plain films or inverter grams or remember that from training. Um And obviously this is a lateral prone X ray performed in a patient whom a fistula has not been visualized in the 1st 24 hours of life. So this isn't something weird Immediately after delivery. We're giving them some time. Um and that's partially to let the arrogant distal and partially to see if officially will develop a lot of times patients who have that perennial um fistula, it will sort of come to a head or you'll start to notice meconium draining within 24 hours Here. The patient is placed in a prone position for at least 15 minutes and that allows the air to migrate distantly, which is possible. And you can see in both of these X rays there's a radio opaque marker where we would expect the normal anus to be. And we can measure the distance between the presumed end of the rectum or where the air column is and the skin. And this relationship between the most distal rectum and skin. And also the relationship between it and the cock six helps us identify patients who may require a diverting toma or we can do a proper repair of their direct a malformation. Um and in a few slides we'll also look at some 18 lateral X rays that we use to measure what we call the sacred ratio. Um and that we use more for prognostication and less for this initial work up and and again how these images and when the patient presents will help us determine the timing of prepare. Um so the other part of the work up um is really getting into the associated anomaly. So keeping constellations of anomalies such as factual and other associated genetic conditions such as trisomy 21 in mind. And so the most important part of this workout will be the Echocardiogram where 30% of patients will have some sort of cardiovascular anomaly. Only about 10% of those though will require intervention. So um important to do the work up but also to acknowledge that it may or may not delay repair of their anal rectal information. If we do find one of these things that tetralogy of the sp something that requires intervention or human dynamically significant lesion. Um It may change what operation we do. So we may be more apt to perform just a diverting colostomy and deal with the leader. Um Next will be renal. Um So gur genital urinary anomalies are described in 30 to 50% of patients. The most common will be vesco, urethral reflux or hydro necrosis. And then you can see here that some of them will also have crypto organism. Hipaa Spadea, Zarina Leigh genesis and dysplasia. Finally, spinal imaging about 30-50% of patients will have vertebral abnormalities. Most common will be tethered cord and that asylum. Um but you can also see more significant lesions such as myeloma, ninja seals, the sacrum butterfly for debray. Um And as we'll get into it as you may know, these affect your ability to have normal bowel function, normal bladder function. So they're going to be very significant in identifying early to help us with prognostication for these patients. So getting into surgery. Obviously I'm a surgery. And so I could talk about surgery and creating these neo anus is all day, but that's not what we're going to focus on here. Um So just briefly, the goals of the operation. Um First is to adequately take down all of the abnormal communications between the G. I. Tract and the G. You track while minimizing morbidity to both and maximizing normal function. Um The second goal is to place the rectum or the neo anus in the correct position, so really to get it into the center of the sphincter mechanism complex that helps us prevent prolapse and then we can recreate a perennial body to separate the anterior structures or the euro planetary system from the rectum. And third is to provide these patients with a repair that hopefully gives them long term control of their bowel and bladder and the possibility of normal sexual function. And we can do this in a couple of different ways. So there's 12 and three stage procedures. Um And the decision making between which of these repairs will do depends a lot on the presence associated anomalies. So whether they have significant cardiac defects, additional intestinal atresia, um and also the timing of presentation. So in our case presentation we had someone who came in late and because they've basically been living with this distal bowel obstruction. Um That's a patient who may require a multistage procedure. Whereas the second patient came in early had a perennial fistula had a good sphincter complex. Had no other obvious anomalies and was able to undergo that one stage repair and then the level of the fistula. So for a patient with a high um fistula who's going to require both an abdominal procedure and a perennial procedure, um they're more likely to get a multistage repair so that they can undergo all the work up and preoperative testing and I'm not going to spend any more time talking about the operation itself. Um briefly some of the complications of the operations um in surgery we sort of divide all our complications into interrupt early post op long term post op. Um so your interrupt complications um that are unnoticed or of significant consequence, unfortunately pretty rare and are largely going to be related to injury to the euro genital system and can include injury to the difference, seminal vesicles, china, um And we do a lot of things during the operations. To try to minimize these things like placing a catheter, um fully doing the repair when the child is a little bit bigger or older, all these things can help minimize the risk of injury. The other thing is that the complications are largely dependent on what surgery is performed. So um and what malformation the patient presented with, so for a patient who does require colostomy creation. The two complications that we sort of worry about most includes prolapse and structure, fortunately these colostomy are usually temporary, making the risks of these a little bit lower. Um But obviously in a patient who has a colostomy has prolapsed, that's a lot for a family to deal with. And if they do require a revision of their colostomy they may lose bowel length and then that will certainly affect the subsequent operation. Um and what we're able to offer the patient and may change things quite a bit for them for patients undergoing indirect capacity. Um In addition to wound complications which are always going to be a risk, especially if the patient does not have a diverting colostomy. Um There's a variety of potential complications shown here. Um stricture in the literature, you can see it reported at around 4 to 5%. There's more recent literature that suggests this is actually closer to 20 to 30% of patients. Um and not really for discussion here, but there's a lot of debate happening in the pediatric surgery world about whether or not dilation help prevent these strictures or change the long term outcomes for them. Um In most pediatric surgeons practices, we will be having the families do dilation and will be engaging in that from a early, early on post operatively a miss location replacement of the neo anus outside the muscle complexes, reporting about 4% of patients. Um and like you said, we talk about the timing of indirect capacity and how this may influence the risk of MS location with the idea being that if we wait for a child to grow, they're more likely to have a well formed muscle complex which will reduce the risk of misidentification of the muscles. This is a very extreme example and what they're showing in the dotted circle is where the actual muscle complexes. And if we stimulated this patient under anesthesia you would see it contract there while their new anus was places quite posteriors. Um The other complications listed here, fistulas um can be divided into the category of things like recurrent, persistent or acquired. Um These are often related to operative technique or injuries to the vagina or your wreaths for making them susceptible to recurrent fistula during the healing process. Um You can also have a missed fistula that's usually related to the assumption that you are dealing with an incorrect malformation without fistula. So in patients who have a colostomy created early on will perform a colostomy graham where we inject dye or contrast into the toma. And if that study isn't done at the right pressure or high enough quality um you may not be able to see that official exists. So um you may have a patient who presents later with recurrent vehicular or with a persistent prolapse I think is probably a little more common depending on how you define it. Um Here again they're saying 1.2 to 3%. Um Sometimes we do require repair and this is just an example of a prolapse. Since you can see it's just some of that um New costa exposed on the one side and so a patient like this with that much um irritation and probably under repair. And so that someone who if they've fallen um fallen out of their follow up with the surgical clinic you may see and refer back for that purpose um and then colostomy closure. Um anytime we do bowel surgery there's risk of leak or stricture complications related to that. So again depending on what operation they're undergoing different complications that we worry about moving along from the operative complications or short term issues issues. I want to focus the remainder of the talk on outcomes I've alluded to. There being some predictors of successful outcomes a couple of times. But one of the things that makes managing indirect amount confirmations um a challenge and that we need to define before describing the factors that predict success. Is understanding how we might measure success in a patient with an indirect confirmation. Um Sorry that came out funny. Um in general we and presumably families think about success of an operation or outcome for an anal rectal malformation as being based on the patient's potential realization of bowel control. And this includes things like their ability to have voluntary bowel movements, maintain fecal continence and also having a low frequency of soiling, having manageable constipation, having appropriate voluntary urinary continence and not having prolapse and there are several tools other that we're using to help us assess these issues which can be challenging given the combination of objective and subjective factors and also the fact that we're performing a lot of these operations on patients who we would not expect to have voluntary about control. So at three months of age when they're done with all the other surgical procedures, you may still have a few years before you have any idea if you have a successful operation in that regard. Um So again getting into sort of these measures of success um for patients may deal with long term challenges related to fecal and urinary continence, um sexual function, quality of life and these things all matter not only to us as surgeons but to the whole care team and the patients and family because as you know, patients continents have a significant impact on their quality of life. Um Again as we're usually doing these operations during infancy, we may have lost the patient to follow up by the time these things are coming into discussion. And so it's important that everyone who sees a patient with this history knows that these things can be related to their initial malformation and requires sort of a more in depth assessment. Um And management plan then one thing that we'll talk about in the predictors is that the distribution of complications and sort of their long term outcomes really vary with the malformation they had initially. So one way to summarize what we've talked about in terms of measuring success is the indirect a malformation continents index or the M. C. I. And what this index tells us or suggests is that as the location of the official moves further from the normal anatomical location of the anus, the associated abnormalities increase in the likelihood of achieving continents decreases. That means if you have that perennial fistula where you have a normal muscle complex and the pain is just a little bit outside of it. Even much better chance of having a normal bowel function and good outcomes than the patient with no fistula or with a bladder fistula. That and extrapolating into measurable um factors three clinical features that we find most helpful in predicting the likelihood of success or the likelihood of achieving continents are the type of anal rectal malformation. The quality of the final corridor associated abnormalities. Something called the sacred ratio in conjunction with the presence or absence of sacred anomalies. So going through each of these, starting with the type of indirect malformation. Um So multiple papers are published in the last couple of decades trying to get at this issue of what makes a successful operation. They specifically look at parameters um such as voluntary bowel movement, soiling, constipation, rectal sensation as well as the need for ongoing therapy or functional issues. Um So in one Patients in one paper sorry that we'll talk about this is a 2017 single institution review of patients with their ends. Um they had 214 patients identified between 2009 and 2016. Um 161 actually met inclusion criteria. And these were all patients greater than three years of age with no known history of developmental delay. I collected data on these patients um comparing three different bowel function questionnaires. Um 80 patients responded to the surveys that were sent out and were evenly split in terms of males and females. And these surveys, I'll get at things like potty training, type of bowel management that's required and also use the type of anal rectal malformation um to help classify the responses. Um what you can see here is that the majority of patients, about 54% were classified as low. So that would typically include anal stenosis, perennial fistulas or um the recto vestibular fistulas and female patients. And when we look at patients who are able to achieve what we would consider full continents, which Pena and described as feeling an urge to have a bowel movement combined with the ability to hold the bowel movement and have volunteer bowel movements. And what you see is that zero patients in um I'll show on the next one. Sorry. Um zero patients with Chloe ca or no official were able to achieve this one. More than half of patients with a perennial fistula had this good outcome. And this means unfortunately that nearly half of the patients who had sort of a good setup or had that perennial fistula and a high likelihood of success still have some degree of soiling or constipation requiring long term care. Um as we already talked about, there's a high association between vertebral or spinal abnormalities and in erectile malformations. In patients with the Bacterial Association, about 51% will have a spinal anomaly and in patients without bacterial um there's still about 30% of patients who will have some sort of spinal anomaly despite not having the whole bacterial syndrome. Um and in a 2015 retrospective review of patients with and without factoring association, patients with the association were found to have worse outcomes with regard to malfunction. Looking specifically at the spine, we know that tethered cord is associated with anal rectal, urinary and lower limb dysfunction and that these symptoms can be progressive. Um We also know that there's a close relationship between tethered cord and an erect a malformation with concurrent Diagnoses and up 30-50% of patients in a study looking at long-term functional outcomes between patients with a history of tethered cord and those without, they found no significant difference. Uh in terms of soil and constipation, involuntary bowel movements. Um This cohort though is unique in that all the patients in this particular study the underwent screening M. R. I. After four months of age, meaning that some asymptomatic patients were identified for inclusion and therefore we may not actually have a good capture on who would or wouldn't have had good bowel control. Um In all of these patients with an incorrect a malformation, we do spine screening early on, which usually includes an ultrasound. Um to look for that tethered cord or x rays. To look at look for normal vertebral and several development. The third component of that indirect amalgamation, continents index or the third factor um helping predict the likelihood of a patient achieving continents about autonomy is called the sacral ratio. This is probably the most often discussed and it's really an objective way of describing the quality of the bony pelvis and sacrum. Um This is not something we necessarily use pre operatively. Um We're discussing the early clinical management, but again it's good for prognostication or sort of long term discussions and what it is is. Um We'll get in the ap and lateral plane films ideally after three months of age, when the bony pelvis has started to classify. And we can calculate this ratio using the distance from the iliac crest um to the posterior inferior iliac spine and divide that by the distance from the posterior iliac spine to the tip of the coccyx and a ratio of less than 0.4 universally incontinence due to that state girl hippo development. While the ratio that approaches one usually predicts a good um prognosis Some examples of how we might use this information. So if patient one has direct a perennial fistula has a normal spectral ratios around one and has a normal spinal ultrasound not showing a tethered cord. And we compare that to a patient with the rectal bladder, neck fistula or a claw waka who also happens to have a hemi sacred and mystical ratio .4 and a tethered cord. We can assume that patient one has a much greater chance of having normal bowel function in the long run. So that information can be useful for us in our discussions with the families and also for us to be able to give you that information as the pediatrician. So you can help the family prepare for what this may mean. Um And again, talking about long term outcomes for these patients, we have issues with incontinence and that can be urinary and fecal, You have issues with constipation, issues with prolapse. And unfortunately these things are all related and all I think somewhat confusing. And even to the general pediatrician, the general pediatric surgeon and the general gastroenterologist, it can be a little hard to figure out what's going on with one of these patients and what their needs are and that's why optimal bowel management really depends on a multidisciplinary team. There's a lot of factors involved in figuring out what's going on with the patient. So the work up and management really important to be involving multiple players. And most um clinics that deal with the bound management for these patients include um Urology, gastroenterology surgery psychiatry, it's usually a pretty involved group physical therapy um And occupational therapy. And this is where I really want to empower you as primary care providers to engage specialists early and often for any patient who you're worried about or who may have been lost to follow up. Um And again things like their sexual history and their ability to have a normal sexual function. Um It may not be the first thing you think about when you think about interrupting information but that may be the patient if they're having issues that needs to be referred back into a program. Um So the assessment for patients with a history of incontinence or constipation usually starts with an exam discussion. And um Oftentimes some plain film x rays and that can tell us whether or not there's a dilated colon, whether or not they have a large stool burden or other abnormalities. Um Occasionally an exam under anesthesia is required. These are usually the younger patients who are having issues where we can determine whether or not that new anus is appropriately positioned if the sphincter complex is intact or if there may be something else going on. So if we think about things that the carina is triad where they may have a pre sacral mass or residual Fishel or something. We may need to identify that in the operating room and then depending on these findings, we can start to create a, bowel management program that works for the patient and the family. Um And so in the case of a patient who appears constipated on imaging and had a good prognosis with a normal sacrum and all those things, they most likely will have constipation with overflow or pseudo incontinence. And these patients can usually be managed with laxatives um or about management program like that for patients with high malformations or poor prognostic indicators or if they have a concurrent tethered cord method, um ninja steel or something else that gives them a low potential for bowel control animals are often required to help what we will say is keep a patient clean or on a schedule. Um It also will use things like the location of the colon imaging to help guide us. Um We're buying a contrast study, for example, in dilated colon in the setting of incontinence suggests hyper motility. So once we rule out a mechanical obstruction or a stricture, these patients may benefit from those high volume enemas. Um equally incontinent patients with a non dilated colon, on the other hand, is more concerning for hyper motility so that those patients may require a combination of bulking agents. Dietary changes in sort of small volume enemas. Um And I know that this seems like it should have been the bulk of the talk, but the truth is like I said, this is confusing for um every level. And so that's why these patients often do require referral back to a multidisciplinary group for management. Um And some other things besides laxatives bulking agents and transitional enemas um are things like the anti grade colonic enemas or ace tubes or tape tubes um where we can provide irrigation zones and um sort of improve propagation of waves through the colon. So really doing that same enema program but um giving them an to grade and this has been preferred by a lot of our patients especially as they start to get older and don't want to participate in transitional um enemas. And then there's also some work being done on understanding what how trans abdominal extra stimulation or T. S. Can be used in these patients to improve colonic motility. And so not as specific to intellectual malformations but sort of these motility issue patients in general. Um And so I know that that was a lot but the conclusions and sort of the take home points in my mind are um one to always check just because the patient is discharged from the nursery and reportedly stooling doesn't mean that they have a normal anus. So do your own exam. Never hesitate to call or refer a patient if you're concerned. Um indirectly malformations with or without syndromes or associated anomalies are really lifelong diagnoses. And while an excellent operation can set a patient up for success. It's not the only or even the most important piece of the puzzle. And so this is really a lifelong problem and not something that we sort of deal with as a pediatric providers and then let go of. Um And that's part of why I'm personally interested in things like transitioning care and how we get these patients connected to the right adult providers to help continue managing these. Um And then last incontinence can mean many things. Um So try not to make assumptions about whether they need bulking or enemas or laxatives and take into account that they probably do require X rays, contrast studies and consultation with Gi and surgery to help determine what their what an individual patients incontinence means. Um And it's not just gonna go to questions but while we do that I'll leave up this slide for how to refer your patients to. UCSF. Thank you Doctor Caro, do you have any more? I'm sorry in front you. No that was good. Um So the there oh if it's okay if anybody has questions, go ahead and put them in the Q. And a Doctor Cairo's gonna get started. She said disregard the above. Um Thank you if anybody has any questions about this lecture. Even any general surgery questions um Please feel free to put them in the talk. Also going to remind you that um a link for the evaluation will pop up at the end of this. Please make sure you um figure make sure you fill those out so that you can get credit for those and this will also be on our med connection page along with other CMS that we've done in the past if you have if you haven't had a chance to watch those you can still get credit for them. And all of our other lectures. Our webinars will be there as well. Um Anybody have questions, any questions on this or any other general surgery questions please feel free to put them in A. Q. And a section dr um For anal rectal malformations, their minimum distance between the vagina and the rectum to measure. That's a really good question. Um And that's one of the troubles that I think people have in the newborn nursery or when patients born. And you're just not sure is that you know it's a little bit anterior or not. And to us it's less about that distance and more about where the muscle complex it is. Some patients will have a sort of anti interior um anus. But if it's in a normal muscle complex we can't make that any better. All we can do is make that worse. Um And so a lot of times patients where it's not clear we'll take them to the operating room just to do an examiner anesthesia and sort of stimulate the muscle to help us determine where the anus is relative to the muscle complex. Um And then a lot of these little babies it will it will look really narrow. Um But if it's in the muscle complex we're gonna call that normal. Um And can you tell from the outside. So yeah so you know in a normal baby probably a centimeter or so it's going to be quote unquote normal but it's really about the muscle complex. Um And more that than the actual size of the perennial body. You may be able to tell some of that without doing an exam under anesthesia. Just saying where there's sort of anal wink and where it looks like there's a normal contraction and how that relates to the anus itself. Uh Some babies may or may not let you do that in the clinic though any other questions and dr kinder. Where do you see patients? So I see patients in Oakland and in Mission Bay. Um As you all know what the merger over the last several years, our pediatric surgery group is really one big group spread across the bay. Um So besides Mission Bay and Oakland at the bending of Children's hospitals. Um Many of our surgeons also see patients in walnut Creek in fremont and um take care of patients at Kaiser Oakland as well. Um And so for me personally it's one of the few who really goes across Bay. Um I can see patients in clinics on either side of the bay and then schedule operations again either side of the bay depending on what works best for the patient and family. Um. Perfect. Thank you. Okay if there are no other questions, we're gonna let everybody have a couple of minutes back their time. Thank you everybody for joining us. Thank you. DR Cairo for a great talk. I hope you all have a great day, and I'm gonna say, end it for everybody by. Thank you. Created by
