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Beyond Typical Childhood Constipation

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  • Interviewer: We are excited to have you join us today as part of our Boys Town physician education series for March. We encourage you all to stay connected with us and take advantage of these free monthly CME opportunities for all physicians virtual style. Before we get started, I'd like to announce that this series is jointly provided by Boys Town National Research Hospital and Creighton University. Today's presenter is Dr. Megan Fuller, board-certified surgeon at Boys Town National Research Hospital. Dr. Fuller received her medical degree from Baylor College of Medicine and completed her general surgery residency at the University of North Carolina Hospitals. She went on to complete a research fellowship in pediatric gastroenterology at Baylor College of Medicine and a surgery fellowship at Children's Hospital of Philadelphia. Dr. Fuller has received advanced training in pediatric colorectal and pelvic reconstructive surgery from Nationwide Children's Hospital. Dr. Fuller joined Boys Town Hospital in 2019. Boys Town pediatric surgery has two clinic locations in Omaha, as well as Lincoln and Sioux City, Iowa. Her special interests are pediatric colorectal disorders, pelvic floor disorders or injuries, anorectal malformation, Hirschsprung disease, chronic constipation, and neurogenetic bowel. Please welcome Dr. Megan Fuller.

    Dr. Fuller: There's many things you could title a talk on constipation over the lunch hour, but this one will be entitled, "Beyond Typical Childhood Constipation." So I am part of a group of pediatric general thoracic surgeons. Oh, [inaudible 00:01:43] them. We think that surgery is always better as a team. And I am part of a great team of pediatric surgeons, my partners Dr. Cusick, Dr. Raynor, Dr. Schall, and Dr. Suh, who have joined us with a specialty interest in pectus and pediatric thyroid shortcuts and cancer surgery. So if you need us, you can always find us with our clinic number. And we do have locations as mentioned, both West Omaha, East Omaha, Lincoln, and Sioux City for clinics. We're trying to improve our accessibility for patients that we know sometimes travel quite a ways. So as she mentioned, this is me. I did med school at Baylor in Houston and residency at UNC Chapel Hill. Go Heels. For everyone following March Madness, I did my fellowship up in Philly at CHOP, and then I did do additional training in pediatric colorectal at Nationwide Children's Hospital with [inaudible 00:0:3:03] Richard Wood. I like gardening and so this is my favorite season. We've got some beautiful weather to start this spring. So constipation, it made an entire episode of "Boss Baby" where Boss Baby can't go boom boom and has to use this first sick day. So we know this is really prevalent. And sometimes it's joked about a lot and it's funny, but it's pretty prevalent, especially in our pediatric and toddler patients.

    And so what is the definition of constipation? So a common definition would be less than two bowel movements a week with stools that are hard, dry, or lumpy in description and stools that are difficult or painful to pass. And as I mentioned, how prevalent is it? Some estimates up to 1 in 20 visits to a general pediatrician. So you'll have to let me know if that follows what you guys are seeing. If we want to use the more stringent definitions of constipation, we can look at the newly published Rome IV criteria, which would be straining during at least 25% of defecations, lumpy or hard stools in at least 25% of the time, sensation of incomplete evacuation, or sensation of anorectal obstruction or blockage, again, for 25% of the time, needing manual maneuvers to facilitate passage of stool, including digital evacuation or support at the pelvic floor, and fewer than three spontaneous stools per week. So, not responsive to an enema or some other intervention. They note that loose stools are rarely present. If you're not on laxatives in children with a diagnosis of constipation, and there have to be insufficient criteria for irritable bowel syndrome. And technically, they need to be present for the past three months with the onset within the past six months. So, it mentions that you have to exclude IBS, which is defined as recurrent abdominal pain for more than one day per week in the last three months. And this should be related to defecation or having increasing or decreasing pain with defecation. It is associated with the change in stool frequency with the onset of symptoms and associated with the change in stool appearance. Depending on the consistency of the stools it is subtyped either into a constipation or diarrheal subtype. There's also mixed-sub types that I'm not going to go into.

    So that's a lot of verbiage. And, you know, really the wrong criteria is utilized for research definitions and people that are really trying to stringently define this. And so, I think the more common definition is a good one and a good pocket definition, which is less than two bowel movements a week and stools that are hard, dry or lumpy, and difficult or painful to pass. And you could add in the feeling of not being able to completely empty with stooling. And so stool consistency is typically described by the Bristol Scale. Also, this is research definitions. And so I like these more common definitions that show you food groups or other things. What better for a lunchtime talk in terms of stool consistency? Three and four, Bristol, three and four are considered normal, which should be formed, but soft. Corn on the cob I actually think is a little bit over-formed but should be more of a soft, deformable consistency. So there should be some form to it. So, no form, so I will often ask, does it have any form when it hits the water? Does it keep its shape, or do you not notice any shape visible in the toilets, or is it more like rabbit droppings and whether or not it hurts when you pass it? And it's really just a surrogate of how long the stool has been sitting in the colon, and how much water absorption has occurred.

    And so what is my definition of typical constipation? I would say this is your usual short-lived, often presenting in toddlerhood constipation that responds to your initial treatments. So your changes in diet and fluid intake activity level may require some stool softeners for a period of time coupled with some behavioral changes, but then you're able to wean back off of stool softeners without recurrence of symptoms. And, you know, really the goal of treatment of constipation, maybe because I just treat complications, is to prevent the complications from constipation, including the development of maladaptive stool patterns from poor experiences with stooling. And this is, you know, your wheelhouse, and these are all of the things that you are treating every day as a pediatrician and you're counseling families on, you know, keeping their legs raised when they stool, trying not to put increased pressure on potty training, potentially putting up pause and potty training where you get it back under control, keeping stool soft, trying to encourage all the normal dietary things that we want to prevent constipation, including fruits and vegetables, which most kids are on a strike.

    So what is my definition of atypical constipation? And so for me, these are kids that have developed complications, if they're constipation, and I'll go over some of those, including constipation requiring to get admission to the hospital. The majority of kids with constipation do not require admission, constipation requiring multiple home cleanouts to get it under control and development of encopresis or fecal incontinence, secondary to constipation. So when I think about constipation, I think about one, is it kind of typical or atypical? What is their stooling history? What did it look like as an infant? Were they breastfed, formula fed? How about that transition to solids? And I kind of want to understand what the pattern is. Does it occur that they do fine, they do a cleanout, and then three months later, they're back in the same spot? Again, I see that pattern a lot in atypical constipators. That's after a cleanout they'll usually do okay for two to three months until they develop a stool burden that is severe enough that they start experiencing symptoms again. And my first step is always to rule out any anatomic causes of constipation, something else that could be contributing to this difficult-to-treat constipation.

    And so many of those things I have listed here. So, you have pelvic or pre-sacral tumors. A large juvenile polyp within the rectum can also cause a mass effect to make it more difficult to stool, anal stenosis, a missed rectoperineal fistula or rectovestibular fistula, Hirschsprung disease or spinal anomalies, or neurogenic bowel. So presacral tumors, no one's going to miss this kid when they show up in your office because they have a clear sacrococcygeal teratoma. But you may miss some of the internal STTs or small other pre-sacral masses, and it [inaudible 00:10:38] that high index of suspicion. And so one thing that may raise your index of suspicion is a sacral defect or a hemisacrum on a plain film. If they have an association of both constipation and urinary symptoms, if they have a diagnosis of anal stenosis or rectal atresia, as they can be present with Currarino's Triad, which is a presacral mass, sacral anomaly, in anal stenosis classically, this is a genetic defect and that makes one gene and can be heritable. And so if there's a family history of presacral masses, that might also raise your index of suspicion.

    As I mentioned, there are multiple etiologies for this, not just sacrococcygeal teratomas. Other germ cell tumors can present in this location, as well as rhabdomyosarcomas, neuroblastomas, lymphomas, bony tumors originating from the lower spine, lipomas, or lymphangiomas. We can also see duplication cysts of the rectum or colon, neurenteric cysts that have a connection to the spinal canal and linings with both neural elements and bowel elements, interior myelomeningoceles that, again, could be missed in screening, and abscesses that could be associated with Crohn's disease or rectal trauma. And so here are a couple of examples of hemisacrums or sacral deformities that were associated with presacral masses. This is the same patient. Sometimes on a regular KUB because of overlying stool, it can be difficult to visualize this space, but you can see that the coccyx and sacrum actually splays off to one side in this picture. This is just a more up-close view where you can see this is post-repair. So there's some surgical material there. And then this is your classic scimitar sacrum where it looks like someone has taken a bite out of the sacrum.

    Here are a couple more patients. And so this was found on a screening MRI. Occasionally, on a spinal MRI, they don't catch the presacral space because they window down so closely along the spinal canal. And so oftentimes in kids that have had screening MRIs, I will go back and look at them and see if that space has been [inaudible 00:13:27] and whether or not the radiologists can comment on it. This was a small teratoma present, and this was a different appearance but also the final pathology of a teratoma. The other place you can see this is on a contrast enema, which you might be getting for ruling out other things within the constipation pathway. And you can see this is this patient here. And there is a widening here between the rectum and the sacrum. Typically, this is actually this patient here. But I would call this a normal contrast enema where the rectum kind of [inaudible 00:14:06] along that corner and you don't have any widening between the sacrum and coccyx and the rectum. So that's just another subtle thing as you're looking at those follow-up studies.

    This is an example of spinal cord tethering, which can also be associated with these presacral tumors. And this is an example of the dural closure and again, a coccyx should be present right here but is not. You can see that this sacrum and coccyx is splayed off to one side. And so I have done this case in conjunction with the pediatric neurosurgeons here and with one of the adult neurosurgeons [inaudible 00:14:53] diagnosis so that they can address the fecal sac and to make sure we have good closure there. And then I address the tumor in the presacrum. That's a great case for the combined approach.

    So moving on to a new topic, this would be a solitary juvenile polyp. And these can sometimes become so large that they actually prolapse out of the rectum and can be confused with rectal prolapse or mucosa and sometimes can become large enough, again, that they cause a sensation of incomplete emptying or a sensation that a child needs to stool when there's no stool presence with the other symptoms that might alert you to needing to look on the inside for this. Anal stenosis can have a delayed diagnosis depending on its severity. Severe anal stenosis may cause inability to pass stools [inaudible 00:15:56] additional studies. But unless severe, a degree of anal stenosis may go unrecognized initially, as the anus is normally positioned. Families of those older kids that are not initially diagnosed may describe spaghetti stools or ribbon stools with straining to pass. Kids may have bloody stools and secondary development of small fissures from the very small opening. And this can be congenital or acquired secondary to trauma. So in the adult literature, they discuss this after surgical procedures and where there's scarring of the anus, but it can also happen if there was perineal trauma or passage of several large stools with development of severe fissures with scarring on healing.

    Again, these anuses are in the correct position. They are surrounded by muscle. It's just that the caliber's too small and narrowed. And they often if you look on the inside have a skin-lined appearance or a funnel anus is the other terminology people will use. Sometimes this is confused with rectal atresia where there's actually an external opening that is blind-ending when you probe, usually just a small fibrotic connection to the upstream bowel. This is typically diagnosed in infancy because they don't stool after birth in terms of rectal atresia. And so in both diagnoses of an anorectal malformation or anal stenosis, it just starts with a good perineal exam. And so things I'm looking for when I'm looking at an incidence of newborn perineum are first just how well-formed the buttocks are. Normal muscles typically have well-formed buttocks and you have an increased suspicion if they have a very flat bottom. If you touch near the anus, you can see an anal wink that can help you see that muscle puckering around the anus to ensure that you're in the correct position. You can't always get it to happen but oftentimes you can on an exam. When you're looking at the perineal body in a female, you're thinking about a centimeter to 12 millimeters in length. And so if that perineal body is shortened, it increases your suspicion that there may be an abnormality. If you do an internal exam on a normal anus, it should feel supple and flexible. You can feel it slowly stretch whereas an abnormal anus will feel rigid, firm, or fibrotic in both anal stenosis or rectal perineal fistula. Normal newborn size if you're using a size Hegar dilator it's around 12. And a one-year-old should be closer to 14 in size.

    The abnormalities we see typically have an opening of around five millimeters. So, really half as large as it should be would be the average presentation. And in case you don't have Hegar dilators in the office, what I recommend is that you just measure. And so Hegar dilators are measured actually in millimeters of diameter, and so a 12 Hegar is 12 millimeters. These are just some examples of a typical thermometer being around four millimeters in size. So if you can fit a thermometer without any resistance then you should keep looking at the size. If you have resistance with a thermometer then you know that you're too small. And then these are just my fingers and so my pinky finger is about a 10 to 11 Hegar. And so I should be able to have no resistance on the majority of newborns unless they're extremely premature or IUGR. And then you can just measure your index finger as well. And mine is closer to a 12 to 14 Hegar. That can give you a good estimate when you're in the office in terms of size.

    And so, then I just have several examples of things that might raise your suspicion. And so this would be a rectoperineal fistula where you can see that the opening doesn't have any puckering around it and it appears to be too small. My guess is this does not feel supple and if you tried a digital exam, it would most likely cause tearing. The other thing you can see is this area behind it with this pinkened coloration that almost says, "I think maybe the anus should be here." And you'll see that on several of these pictures. This would be a female, and you can see that the opening is within the introitus. So the labia are ending here, and the opening is within it. And so this would be a rectovestibular fistula. You can see the vaginal opening here and that there is essentially no perineal body present. You can see also, it looks somewhere in here would be visually where I would expect to find the muscle complex, but pretty well-formed buttocks, as we often see in the malformations.

    This would be another example of erector vestibular fistula, but where you can see a more intact time in here. Again, there's really no perineal body. And that opening is within the introitus. In an older kid where this may have been missed due to stooling over time, this opening may not be small in caliber because they have slowly stretched it over time. This picture raises suspicion because there's a very short perineal body. But what I see in this picture that I haven't seen in the other pictures is I see muscle puckering circumferentially around this penis. And so this is a normal anus that just happens to have a short perineal body. It's surrounded by muscle, and nothing I do surgically will make this function better. This may be a child, due to the confirmation of where this anus is located and those recto anal angles, that may be a little bit more prone to constipation, but nothing surgically I do will make it better. This is another example of a recto perineal fistula where I can see just a little bit of meconium here present. And this is a good example of a very flattened bottom despite the presence of appearing of fistula. This is an older child with a recto perineal fistula and they're even trying to pass the stool and they are straining and straining and straining and they are unable to pass it. And you can see this area just behind it that has that same slightly pink, almost mucosa-appearing area. And so this was an anus that on stimulation had no muscle, essentially, from the forward three-quarters of this. And there was a little bit of muscle here that should have been the anterior limits of where their anus was. And so this was a good pickup later on in life.

    So what are those other conditions that might lead to constipation, Hirschsprung disease, which we do a lot of rule-outs for? Because I really can't tell you someone doesn't have Hirschsprung disease without a gold standard biopsy? And so, in kids that have struggled for years, it's good to know that sometimes they just don't have Hirschsprung disease. We don't need to do anything different in terms of their management. They will have a similar rectal exam to anal stenosis at times. Their anal canal can feel tight, but then it opens up to a normal bowel caliber. They'll admit a normal-sized Hegar but often there'll be increased resistance to passage. Although there's not commonly tearing like you might see in an extremely narrowed anus like you might see with erectile perineal fistula or with anal stenosis. These anuses are in normal location. Again, it's the lack of these ganglion cells, so the colon doesn't relax the way It should in normal peristalsis. And via anorectal manometry, we can see a loss of the rectum annual inhibitory response and I'll show you some pictures of that.

    Delayed diagnoses often present as the kid's transition to solid foods that are more difficult to pass. And the characteristic stenosis of the distal part of the bowel or the aganglionic part of the bowel also seems to worsen with age, which may exacerbate their symptoms. There's definitely a lot we don't know about Hirschsprung disease. Why some kids get enterocolitis and some kids never get enterocolitis would be a good example of that. And those with delayed diagnosis often are the subtype that are not Crohn's or enterocolitis, despite the bowel obstruction present.

    And so, this is just a reminder of the anal canal. This would be a coronal view of that. So distally, we have an anal canal that is squamous lined. And then we're having a transition to a new site here at the dentate line. We have a lot of sensory fibers in this area, as well, that help us determine if stool is formed, or if there's gas present. And some kids with very poor muscle tone at the floor of the pelvis, such as myelomeningoceles, especially newborns that have poor tone and haven't had time for those accessory muscles to develop increased strength, you will see a flattening here of the anal canal. And I think I've been able to see, like, a visible dentate line where it's not really true rectal prolapse, but it's like their bottom is completely flat and this outer portion is just flat because these muscles are essentially flaccid.

    So when we're talking about the sphincter muscles, we have the internal sphincter muscles and the external sphincter muscles. The external sphincter muscles are under voluntary control. The internal muscles are autonomically controlled. And so we test this by inflating a balloon within the rectum, an anorectal manometry. And you should have a feedback loop that causes relaxation of these internal sphincter muscles automatically. And then in a normal feedback loop, your head picks up and says, "Oh, I gotta go to the bathroom." And then, your external sphincter muscles contract so that you don't go to the bathroom on the floor right there and you'd have time to make it to the bathroom. And so this is an example of a normal reflex where they inflate a balloon within the rectum and you see decrease in the pressure of the sphincter muscles. And this would be an abnormal example where you inflate the balloon and you actually see an increase, definitely not a decrease, in pressure of those muscles. You can get an abnormal response in kids who've blown out their rectum such that they've lost sensation from overstretching in that we can't really get a balloon big enough to elicit a response. And so those kids all need verification with a biopsy if there's a lack of response, as in this picture.

    This would be an example of a delayed diagnosis. This was a two-year-old who had had failure to thrive would be his overall presentation. He presented with a microcytic anemia with a growth percentile less than a third percent, even when he was completely full of stool. This was a screening X-ray secondary to his distension. And then this was his contrast in the map, which may also help us diagnose Hirschsprung disease. I see, again, I follow that curve. So I don't really have high concern for a presacral mass. It doesn't 100% rule it out. And then I see on this picture both contractions of the rectum, which are abnormal. It should relax and stretch. So, here, and then the other thing I see is that the rectum is much smaller than the sigmoid colon, which also is backwards. The rectum should stretch. That's its job. It doesn't contract. It stretches and holds stool and the sigmoid's job is to contract. And so, it's gotten blown out over time. His biopsy showed no ganglion cells, and his level as expected was low based off of this contrast [inaudible 00:29:39].

    So neurogenic bowel is a bowel that doesn't respond appropriately because of a spinal problem or a more peripheral nerve problem. So we'll see this classically with spinal cord injury patients or those born with my myelomeningoceles. Oftentimes, we see this accompanied by neurologic difficulties as well. And you may have some of this subtype with symptomatic tethered cord. Really, the problem with neurogenic bowel is twofold. Both, you can get colonic dysmotility. And usually, that's hypoperistalsis and slow transit intrinsically, and you can also see disordered pelvic floor contractions. And so this was a good review out of a PM&R text where this is, like, your classic muscular control of stooling. And so you have your puborectalis sling, which relaxes when you go to stool and allows this angle to straighten. And so normally, you have your sensory perception, just like we talked about where your body senses something within the colon. And then, it causes those internal anal sphincters to relapse. And then, you consciously relax the external sphincters and the puborectalis muscles, and that angle straightens and it allows for passage of stool.

    You can get incontinence in these conditions where you have low resting pressures, weakness of the muscles, and neuropathy from both altered rectal anal sensation and diarrheal conditions. So this is your classic myelomeningocele, treated with myralapse [SP]. They don't have sensation of the liquid stools. And so, myelos that might have some continence will do really poorly with liquid stools. And if they get sick and get put on antibiotics and their transit increases, you'll hear that they're having leakage.

    This is similar to kids with anorectal malformations. They also have altered sensation and do better with formed stools, and will struggle more when they have liquid stools. And you may see them get incontinent when they develop liquid stools. And then you also have the opposite problem with spinal injuries, at times, that you'd have both prolonged colonic transit, and you actually have an increase in these muscular contractions where they don't relax appropriately. It also makes it harder for them to be able to pass stools. And so kind of just thinking through what those different factors might be for each kid can help you find a way to treat their constipation more effectively.

    So next, I wanted to just talk about complications of constipation. And so these are those things that you start seeing mount with the development of hard stools constipation over time. Hopefully, we can interrupt the cycle of constipation before these develop. The first is anal fissures. And these are, you know, classically bleeding with sharp burning pain with passage of stools. It can also cause pruritis as they're healing. And so you may hear that symptom as well. If longer standing, you can also see the development of skin tags or heaped edges around those areas of fissure. They may be visible on external exam if they extend out onto the squamous-lined portion of the anal canal. That can also be present just on the inner image or not visualized on external exam without spreading.

    First-line treatment of fissures is really stool softener, symptomatic control, trying to prevent further trauma to that area unless the fissures have developed secondary to massive diarrhea, in which case you need the opposite treatments where you don't work hard stools, but you wanna try to firm the stools to decrease the frequency. And we see this sometimes in kids with a short bowel and frequent stooling, Hirschsprung disease, especially total colonic, and some other conditions. You want to screen for underlying causes of fissures. And it's not that you necessarily have to order additional studies but you kind of want to think through if this looks like a classic fissure or if there's something abnormal about it. As an adult, anal canal cancers could present this way. Infections, IBD if they're severely associated with abscesses. And also you want to think about trauma and non-accidental trauma as well, kind of overstretch injuries not secondary to overstretch from passage of large hard stools.

    Warm soaps can help relax the muscles of the pelvic floor and decrease the pain with passage of stools, as well as topical lidocaine. If it's a larger child, you can also try topical diltiazem or nitroglycerin to relax the muscles of the anal canal. And then if that's ineffective, or they have severe disease, we can consider injection of Botox to the sphincter muscles to relax the muscles, which helps the pain very quickly, and is very effective in promoting healing of the fissures. But then we still have to address underlying constipation to prevent a recurrence.

    Hemorrhoids are classified as internal or external based on their origin above or below the dentate line. Those that are present above the dentate line are the ones that you hear about that are amenable to banding that have a narrow base and you can get prolapse of this tissue outside of the anal canal. You can also have mixed hemorrhoids of both internal and external origin. And these are from venous congestion or stasis, and they can be secondary to constipation or straining over time. We also see hemorrhoids more commonly in patients with liver failure or pelvic tumors. So again, just watching for any other warning signs that point to something other than chronic constipation being our cause. Treatment is very similar to fissures. You can add topical steroid cream for short bursts of time. That can help symptomatically quite a bit. Thrombosed external hemorrhoids. If they thrombose within 72 hours just in straining, the blood clot out may help with symptoms, but they will recur. And so those are hemorrhoids that might require a surgical intervention.

    Surgical removal is really reserved for those with symptoms. And chronically, those symptoms are commonly inability to maintain hygiene, kind of chronic bleeding, and mucus production that becomes problematic. Difficulty with wiping, kind of stool streaking because you're unable to clean well. Surgical removal is painful, even with long-acting agents. And so I really want to make sure that there are no other options in terms of treatment and symptomatic control before we remove them, especially in kids.

    Rectal prolapse. You get spontaneous reduction versus manual reduction is one of your key questions to ask whether or not after they stool, it immediately pops back in, or if they actually have to press it to get it to reduce, or if it's irreducible. And those are typically presenting to an emergency room, or if you've tried to reduce it and it's unable to reduce. These we will see sugar placed on and, really, the goal there is to decrease the swelling or edema within the bowel wall. Salt would probably also work. But, again, salts on a wound hurts. And so, that's like classically people have used sugar to try to decrease that swelling and help with reduction.

    We can see rectal prolapse with both chronic diarrhea and chronic constipation. So again, you want to treat the underlying condition. And if that's chronic constipation, it's softening the stools and decreasing the straining time is really what we want to address. And the majority of these kids also benefit from pelvic floor physical therapy to help them understand how to relax the proper muscles and to have the correct muscles contract when they're trying to stool. And that will often be helpful. Unless there are severe complications, I recommend six months of pelvic floor physical therapy prior to surgical intervention. And I have had several kids where the prolapse resolves, and we've been able to avoid surgery. If it persists, there's several different options. I favor a laparoscopic rectopexy where we attach the redundant rectum up to the anterior fascia of the sacrum to help prevent further rectal prolapse. There's some pictures of pelvic floor disinertia [SP] coming, and then you can see why that operation might work. But again, I have to prevent constipation, or we'll be right back where we're at. And over time if they continue not stooling effectively they can re-prolapse despite any of the surgical interventions.

    Stercoral ulcers are best thought of as internal pressure ulcers and if they're bad enough, they can actually lead to perforations. And you can picture this as just a very hardened stoolball that's sitting there and decreasing the blood flow to a portion of the colon. These can become chronic with bleeding and other complications. And so, then first line is just constipation, and then if they have been so long-standing that the portion of the [inaudible 00:40:25] is compromised, it may require bowel resection of that area, again, the treatment of [inaudible 00:40:29. Withholding behaviors. So, this is usually a cycle that starts with passage of a hard stool. And essentially, toddlers primarily is where you see development of defecation anxiety. And the cycle worsens the underlying constipation as they withhold. And so, then, the stools get harder because they're not passed as quickly. And that reinforces the behavior. And so, this is where really trying to treat these kids early, hopefully, helps prevent this cycle from getting too ingrained. The other thing to think about is presence of a neurodiverse diagnosis, and that we do see constipation more commonly in kids of neurodiversity. And it may be that this cycle is more prone to develop, or that they have altered muscle sensation. I've always thought that there is an unknown connection between the gut and the mind because, so oftentimes, we'll see a problem in the brain leading to constipation that's not a classic spinal cord injury or other things. And so trying to think through this with families and again, treating it early to try to prevent these withholding behaviors.

    Pelvic floor dyssynergia is due to poor muscular tone or increased muscular tone and uncoordinated relaxation and contraction of the muscles while stooling. We see this in a majority of adults with chronic constipation. And it can be diagnosed with awake anorectal manometry, or defecography, where they actually look at that passage of contrast as a surrogate for stool passage. I'll show you some pictures of MRI defecography. This would be normal stooling. So, at rest, you can see this is where that puborectalis sling sits. And then with a squeeze, you can see that I have got some increased pressure within the distal rectum. And then with straining, I'm relaxing that puborectalis, and that angle is straightening to allow passage of contrast. This would be an example of intussusception, which is kind of the step prior to the development of rectal prolapse, where as they start, you can see this development of an angle here. And you can see that telescoping of the bowel down within the distal bowel. This would be another example of that where it's more progressive, and you actually get rectal prolapse. So again, at rest, and then, with defecation, you see the start of intussusception, and then that intussusception progresses until the bowel actually externalizes.

    This would be an example of a spastic pelvis, where this is at rest. And then, here is where they should be relaxing that pelvic floor and you should start seeing passage of contrast. And instead, this angle actually tightens. It doesn't relax at all, preventing them from being able to pass the contrast. So that's this condition here with a spastic pelvis, whereas this would be what you see with normal [inaudible 00:44:08] application where that angle straightens.

    And so I think this is really underrecognized in kids, as they have to be cooperative to be able to obtain these studies. And so in kids that are really struggling with a sensation of incomplete stooling, rectal prolapse, or behaviors that sound like withholding behaviors that can also cause, down the line, this spastic pelvis, I will refer to pelvic floor physical therapy, even without the confirmatory studies. Because most of these kids are also anally defensive would be the term that my mentors coined. And so if they're anally defensive, you also can't get the definitive studies to help you diagnose pelvic floor to [inaudible 00:44:56].

    And so really, why do I care? Because really, my hypothesis is that early intervention will help prevent complications. And if you catch it early, hopefully, you can treat it and recognize those kids that are failing, catch any of those anatomic or other pieces. And some of those are psychosocial and family pieces that also need treated to help these kids stool effectively with really the goal to help kids lead full lives. I have a plug for the PCPLC here, which is the Pelvic Pediatric Colorectal and Pelvic Learning Consortium of which I and Boystown is a member where we have multiple institutional studies looking at how we can better treat these kids. Because it's really been a lot of single-institution studies, an under-researched condition, I would say this is maybe one of the next healthcare epidemics. As we think about public health and obesity, I think constipation has been on [inaudible 00:46:00], probably from our Western diet and a lot of other things. Because although this is, you know, the sexy part of colorectal surgery, perhaps, where I get to think through complex reconstructions for things like inter-rectal malformations with the cloaca, it is far less common than the 1 in 20 visits to the pediatrician for constipation. So that's really, why I care about constipation.

    Bowel management is really what do we do. It's really to support kids with atypical constipation and incontinence. And so, once you get to that, beyond typical constipation, or if you just have a question we're always happy to talk through. But some of these other conditions have some wrinkles in how do you manage a kid's constipation or incontinence. And they might require some different interventions than your standard to ensure success. And then I want to be able to support these families because I want them to hit all of their important milestones. If this is a constipator prior to toilet training, I want to get them to that point where they're toilet trained in social incontinence. As things change in their lives like puberty that might affect their continence again or their bowel motility, I want to make sure that they make it through those milestones successfully. In kids with anorectal malformations, I want to make sure they have been transitioned to an adult provider to help them with decisions like childbirth and prevent any secondary complications.

    We also see school attendance as a huge issue with these kids. We see a lot of chronic absenteeism. And school success really starts with being in school first. And so I want to keep these kids in school, improve their quality of life. I want to remove the stigma for these kids related to poor bowel control and the inability to do the things that they want to do in their lives. And so again, my definition of atypical constipation would really be those kids with developmental complications of constipation requiring admission to the hospital, multiple at home being outs, development of encopresis or fecal incontinence. Those kids that get into those patterns so that every three months having a problem and constipation with underlying anatomic abnormalities.

    And is there success in these kids with bowel management? There is. There's a lot of good studies out there showing that you have improvements and you decrease both hospitalizations and ER visits for future constipation with bowel management. Most of the follow-up is short-term and most kids need long-term treatment. In those kids that are atypical constipation, you also want to think about weaning your intervention slowly and making sure families have a rescue plan so that they don't leave your clinic and then two years later, come back with issues that are more severe than they started with.

    This is our team that helps with bowel management and evaluation of these kids. Dana Zortman, who is our colorectal nurse coordinator, and Esther Nolasco. This is my cell number if you ever need to contact me or my email if you have any questions, and then our clinic number, which should be able to get us 24 hours a day. We roll over to the answering service at night if you have questions overnight, or things that we could help you address.

    Interviewer: Thank you, Dr. Fuller, for a great presentation. We did have a question that came through. It is," What is the correlation between colic and constipation?" And the second part is suggested treatment of colic constipation.

    Dr. Fuller: So, I think you're referring probably to infant's dyschezia. Feel free to type in if I'm going the wrong direction here. I think for some infants that sit and strain and turn red, I wonder if it's not the very first manifestation of a pelvic floor dyssinertia [SP] where they just haven't quite put two and two together because we see them where they look like they need to stool and then they're straining and straining and screaming, and nothing is passing. I have had some infants, I usually will try to wait until they're more in this six-month timeframe, that with the addition of a very small dose of a propulsive, like Astana, I've used in smaller children than I have [inaudible 00:50:35] have helped them be able to pass the stool without the screaming and the straining when it's persisted beyond that kind of six months of age. I had some families that that's been helpful for.

    Otherwise, I think it's just, you know, looking at that stool caliber. Some of these are, you know, formula-fed infants that are having really hard and frequent stools. And so, we are trying to treat that underlying as well and giving families kind of a backup plan if they hadn't passed a stool in several days because sometimes, in those that are more constipation related, we'll see a spike in those symptoms when they've gone multiple days without stooling, even though we know for some infants that aren't straining and turning red, that that can be within a normal range. So hopefully, that helps answer your question some.

    Interviewer: Great. We did have another question come in Dr. Fuller. It is, "Can you touch on the correlation with anal dilations and psychosocial issues?"

    Dr. Fuller: In terms of standard anal dilations after, like, an infant surgery is what I think that's probably referring to, there's actually some emerging data after anorectal surgery where we have classically performed dilations that it may not prevent strictures the way that we thought previously. And there's some multi-institutional studies ongoing. Because when you talk to families, they do support groups for kids that have had dilations in the past, when you talk to families about their surgical experience, the thing that gives them the most PTSD are anal dilations. And so, if we don't have to do them, then we usually try not to. One of the conditions that anal dilations are difficult to get around is anal stenosis. That can be both diagnostic and therapeutic.

    And so, I try to minimize the number of times and the length of time that we do dilations for it to maintain a caliber in anal stenosis. And in older kids or delayed diagnoses, I will try to prevent having to do dilations routinely. I do think we definitely see withholding in some of the symptoms and kids that have had abuse histories, which also makes a lot of sense. I do think that I do have kids that have had anal dilations with good parents that talk it through and don't get really anxious about it. And I think there's also a piece of how it's performed and interacting with that child. So I wouldn't say it's 100% of the time in kids that had anal dilations that they develop poor stooling patterns.

    Interviewer: Dr. Fuller, is there anything that you would like to share in closing?

    Dr. Fuller: I mean, I just think if you guys have any questions at all, feel free to reach out. If it's a perineum that you're just not quite sure about and want to get a second set of eyes, we're always happy to do that. I definitely have infants that, you know, were the classic premature baby that had difficulty stooling, and we rule out anything bad. And we just basically send them back with a rescue plan for down the line and a number if they develop problems later on. So we're happy to help as a resource. And we try to do everything as much as we can for families that are from far away close to them in terms of bowel management. As opposed to making them come to Omaha for a week and get X-rays every day, we try to do it remotely as much as possible to keep them in their home environments with their home diets and with their home people. And so, I think that's that.

    Interviewer: Great. And thank you for the submission of questions from our attendees. I'd like to thank Dr. Fuller for your time and expertise today. Again, we encourage you all to stay connected with us and take advantage of our free monthly CME opportunities. Watch for follow-up email communications announcing our upcoming presenters with the Boys Town Physician Education Series, or visit our website @boystownhospital.org. Thank you for joining us today.​


Physician Education Pediatric General and Thoracic Surgery