So in order to start, Doctor Pena is gonna give an introduction of overview of anorectal malformation, and I want you to keep in mind that even though we love to discuss operations because many of us are surgeons, today we're gonna focus more in the treatment of fecal incontinence and constipation. So even the talks about an ectum malformation or histro they will be towards that portion of the treatment. Thank you and enjoy. Good morning and good afternoon and good night to everybody. Um, since we started operating on children with anorectal malformations in 1980, we very soon we learned that um even when our results were much better than with the old traditional techniques. We were far from solving all the problems. A good number of patients had excellent results in the repair of anorectal malformations and had bowel control. In fact, around 75% of all the patients that we operate do have an acceptable bowel control. They are never perfect. But there is a significant number, at least 25% of the patients that are born with malformations that are very complex, very severe, and we have not learned how to create nerves, how to create muscles, and those patients. are so far suffered from permanent fecal incontinence and the question is what to do for them. Should we traditionally the surgeon performs an operation and then send the patient back to the pediatrician and we believe that's not correct. We believe we should do something for them and um and the, the cost of this management is rather low. What it requires is a lot of love and dedication. Um, to be successful. So we believe, as Doctor Bishop said that we can benefit thousands of children with a relatively minor effort using our bowel management program. We have been very much interested in the last years in trying to determine the future functional prognosis for each child born with anorectal malformations. And because we have been following our patients for a long time, we believe that we have learned uh for the long-term follow-up that we can determine the functional prognosis in in the first few days of life. By doing that, we can adjust the expectations of the parents. We don't create false expectations. The parents have a child with anorectal malformations, they believe that the operation is going to make him normal and that's, as you know, is half true. So that's why today, uh, I'm going to be talking about. An overview of anorectal malformations but with emphasis in the functional prognosis based on our own experience. So we encourage you when you deal with a baby with anorectal malformation in the first few days of life to concentrate in trying to determine what type of malformation and what is specific functional prognosis for bowel control, for urinary control, and for sexual function. If it happens that the baby belongs. to the bad side of the spectrum, then you have to, to suffer with the with the family and tell them your baby will have no bowel control, but we will be there by the age of the the child has to go to school, we commit ourselves and we promise that we'll keep your child completely clean so he can go to school, not being discriminated and be accepted in this society. So that's the purpose of this bowel management program. So, um, we are going to start in the spectrum of anorectal malformations from the simplest of all defects is what, what we call perineal fistula, and you see in your screen a male patient with the opening of uh of the rectum a little anterior to the center of the sphincter. We call that perineal fistula. We do not use the terms anterior anus or anything like that and uh. The these patients have an excellent functional prognosis. In our experience, 100% of these patients have bowel control by the age of 3, but instin paradoxically, being the most benign of all malformations is the one that that that suffers from more constipation. Patients, the good news is that these patients have 100% chances of having bowel control, but the worst constipation occurs in this particular group, and you must be aware of that to treat the problem of constipation because we want you to remember always that constipation produces fecal incontinence. Now, 100% of these patients have bowel control provided they have a normal sacrum. Most of them have a normal sacrum. And some of them have a pre-sacred mass and a presacred mass in a hemisacrum changes completely the prognosis and that's why it's very important to take an AP film of the sacrum and it turns out that presacred masses are more common in these defects. Next, in the, in the female, we see the same malformation we also call perineal fistula and the anal opening is located a little anterior to the center of the sphincter. These patients will suffer from constipation and the constipation is incurable. but manageable and it's very important to tell the parents and we like to make the parents of our patients paranoid about, about constipation, so they can be aggressive in the management of constipation, otherwise these patients develop overflow pseudo incontinence. The operation is simple, but remember these patients are going to have Um, constipation with and without an operation. We recommend the operation as if the babies are born in our hospital, we offer the operation in the first few days of life, and the operation consists, as you know, in moving this, the, the anal opening back to the center of the sphincter. Some families decide not to operate and we respect that because, but, but then the patient needs anal dilatations because usually the anal opening is too narrow. Next. This shows you the perineal fistula in a female patient. You can see, you can see there the, the, the orifice located between the female genitalia and the center of the sphincter next. So in summary, a rectal perineal fistula is the most benign type of an erectal malformation, has a 100% chance of bowel control provided the patient has a good operation. Because the sad part of the story is as we are talking right this morning, in some place in the world, a baby born with a good prognosis malformation is receiving a bad operation and therefore is going to suffer fecal incontinence, not because of the malformation, but because of a bad operation, but that's another story. So they provided the patients have noted the cord, that's why all babies should have an ultrasound of the spine in the 1st 3 months of life and provided the patient has no presacred mass. The next malformation that you will see that you see in your screen is called rectal vestibular fistula, and the rectum is This opening in the vestibule of the female genitalia next to the vagina. It is not a vaginal fistula. A lot of people get confused. This is by far the most common defect in females that we have seen, and 95% of the patients operated by us with good sacrum and not at the cord do have bowel control. 70% of them have constipation, so. This operation is, is a delicate operation sophisticated because as you can see in your screen. Rectum and vagina share a very thin common wall, and the real challenge of the operation, regardless of what technique you use consists in making two walls out of one. And moving the rectum back to the center of the sphincter. So we put a lot of emphasis in in the trainees in pediatric surgery all over the world because this is the malformation that all of you will be seeing this week and, and uh if you do a good operation, these patients will become basically normal females and from the sexual, from the urinary point of view and from the bowel function point of view. So rectal vestibular fistula is the most common and rectal defect in females, a good prognosis provided you do a good operation and the patient has a normal sacrum, not at the cord, but remember 70% of them will have constipation. Then there is one particular defect very interesting that only happens in 5% of all cases. That's the anorectal malformation without the fistula. Half of these patients are Down syndrome, and if you take the Down syndrome babies with anorectal malformations, 95% of them have this specific type of defect. A lot of doctors believe that because the baby has Down syndrome, deserves a permanent colostomy. We don't agree with that because 80% of our Down syndrome with an rectum malformations get bowel control. When babies with this malformation without Down syndrome. 90% of them in our cases have bowel control. Remember, when we say bowel control, we are talking about voluntary bowel movements, they are never perfect. All patients with anorectal malformations have two enemies. One is constipation and the other is diarrhea. If one of you, one of us has a severe episode of diarrhea, sometimes it's difficult to reach the bathroom. For patients with anorectal malformation, most likely they will not reach the bathroom. So they should, should be very careful to avoid diarrhea and constipation not treated well will produce overflow pseudo-incontinence. Next, yes, the, the uh next. Then the next malformation in in male patients is called rectal urethral bulbar fistula. The rectum, as you can see in your screen, is located in the bulbar portion of the urethra. Remember, we don't use terminology such as high, intermediate, and low because Mother Nature doesn't work that way. Mother nature works creating a space. Spectrums in everything that they do, everything that happens in nature. So in these patients, the rectum opens in the lowest part of the posterior urethra. It's the most common defect that we see in male patients. 85% of our patients have bowel control, provided they have a good operation, good sacum, not. Cod and the operation that we use as you know is a posterior sacs and the recoplasty, and we believe that laparoscopy is contraindicated in this particular type of defect because we are seeing more and more posterior urethral diverticulums left due to the incapacity of the laparoscopies to reach that lobe in the pelvis. So they remember these patients 85% chance of bowel control provided they have a good operation, not as the core, good sacrum, and the majority of patients will suffer from constipation. The next malformation is recoprostatic fistula. Here, we're getting into the more serious kind of uh type of malformations. The prognosis is not as good in our hands. 60% of our patients have voluntary bowel movements by the age of 3. So, that's not a good number because it's almost like flipping a coin and the parents suffer a lot when we tell them that the baby has 60% chance of having bowel control because they don't, they don't know what to do with those numbers. So when the baby, but when the baby reaches the age of 3, if the baby doesn't have bowel control yet, it has to go to school and it's everybody is totally trained except him. Then we offer them the bowel management to keep the patient artificially clean, so he can go to school and nobody knows about the problem and but then every year we ask, we, we ask the family to come back with the child, so we can try to stop the enemas and see how much bowel control the patient gets because the patient has potential for bowel control, remember, 60% chance of bowel control provided. We do a good operation. The patient has normal sacrum and no evidence of death cord. Some of the, these patients we operate posterior side, but also in some of these malformations, laparoscopy is could be good if you are a good laparoscopist. And then the highest of all defects in male patients is the so-called rectal bladder neck fistula. The rectum is connected to the bladder neck. It's extremely high. Fortunately, these malformations only occurs in about 10% of all cases, but unfortunately, about only 20% of our patients have voluntary bowel movements by the age of 3. This is the ideal case for laparoscopy because the, because it's very easy, #1 because it's very easy to reach from above and #2 because if we, if you don't use a laparoscopy, the patient needs a laparotomy, and number 3, because the, the connection between the rectum and the bladder neck. Doesn't have a common wall. In other words, you can go there and ligate the fistula literally. So lie trying to ligate the fistula in the rectal prostatic or bulbar may cause a lot of damage because they have a, they do have a common. Whereas as you can see here, the rectum is connected to the bladder neck in a, in a, in a way that is described like a fistula and you can put a tie laparoscopically, but the prognosis is not good regardless of what technique you use and, and, and of course if the patient has a bad sacrum, the prognosis is even worse and in this spectrum of malformation that I show you the percentage of associated defects. Runs almost mathematically the same as this. In other words, perineal fistula is about 50% chance, 15% chance of having associated effects, whereas bladder neck fistula, 90% chances and everything in between the spectrum. In other words, you have to be more and more concerned about associated defects, mainly urological, second orthopedic, and then gastrointestinal, the higher the malformation. Then then we are going to the the territory of Cloacas. The um in 1982 I we use for the first time the posterior factor approach for this malformation and of course since we are very concerned about the, the trying to establish the functional prognosis for these patients in the malformations that I discussed, we are mainly concerned about bowel control and sometimes urinary control. In Cloacas we are concerned about bowel control, urinary control, sexual function, and the chances of the, the patient to have. Babies in the future, is it, is this, is this patient will be able to have sex? Would she be able to become pregnant? Would she be able to deliver vaginally or by cesarean section? Those are our main concerns, and it took us a few years to learn that, um, yes, we can determine that in the first few days in, in life. We based on, on an experience of over 570 cloacas, uh, we can tell you that. The sacrum is still extremely important. Bowel possibilities of bowel control depend very much on the quality of the sacrum, characteristics of the sacrum, and then urinary control depends more on the length of the common channel, which is the distance between the external opening of this, um, malformation to the point where the rectum, vagina, urethra bifurcates or trifurcates. So, we have learned that the turning point seem to be around 3 centimeters. Shorter than 3 centimeters, 70% of our patients have urinary control. Over than 3 centimeters common channel, 20% have urinary control, the other 80% need intermittent catheterization to empty the bladder. So, and we also believe that when the patients have a common channel of shorter than 3 centimeters. We can train young pediatrics, general pediatric surgeons to repair the malformation because it is, is, is the malformation is repaired with an, with an operation that we consider reproducible, whereas when the common channel is longer than 3 centimeters, we believe. That first, there's not enough cloacas to train everybody and second, the malformation is very complex, so the decision-making algorithm requires a lot of experience not only in pediatric surgery but also in pediatric neurology. So, um, the bowel in, in summary, the bowel control, the prognosis for bowel control depends on the characteristics of the sacrum and the prognosis for urinary control depends on the length of the common channel. And here you see a cloaca with a long common channel and then of course uh there are many other things that we could discuss about cloaca that will take us an entire day, but today we are only concentrating in the, in the, in to try to establish the prognosis in these babies, and this is something that you can do in the 1st 3 days of life. By looking at the perineum, by, by taking an extra theme of the sacrum and the lumbar spine, an ultrasound of the spine, lumbo lumbosacral spine to determine whether or not the patient has test cord, and just by doing that you can be fairly accurate in trying to establish the functional prognosis of the patient. Now, now we are getting to, um, the most complex perhaps of all malformations that we see. Fortunately not very common is what is called cloacal atrophy, as you know. And these these terrible malformations, the, the patients have the pelvis open anteriorly like a bladder atrophy, but in addition, they have no anus, and as you can see in this, uh, in this picture, they do, they have two hemibladders, and in between the two hemibladders there is a bowel protruding usually like an intersusception like prolapse, there usually is a piece of small bowel or sometimes colon. These patients um will have a terrible quality of life, doesn't matter what you do, but it turns out that they are very intelligent, most of these patients that we have been exposed to and very charismatic and lovely children. Sometimes they have serious spinal problems, sometimes they cannot walk. So the cha the, the, the chances of bowel control for these patients is, is not very good. About 15 or 20% of our patients have have voluntary bowel movements. Most of them don't have, and most of them would require bowel management. Therefore, urinary control is out of question. They will need intermittent catheterization for life because they most likely, most of them need a bladder reconstruction and a mitrofanil. So, the other problem, the, the, the, some of the patients they, they would never even have a pull-through because Most, most of these patients have different degrees of shortness of colon. So that means if if if a human being has no colon, you know that that person is going to have liquid stool for life. Bowel management cannot be done with liquid stool. Patients incapable of forming solid stool cannot have bowel management and therefore the most important formal contraindication for a put-through is incapacity to form solid stool. There is a misconception all over the world. Many surgeons don't perform a pull-through because the patient has meningocele. Don't perform a pull-through because the patient has a bladder neck. Don't perform a pull-through because the patients have the cord. We don't consider those contraindications for the pull-through. If the patient is. Capable of forming solid stool by definition is a good candidate for, for bowel management. On the other hand, even if the patient has good sphincter, if a patient has no colon, there should never have a pull-through because the bowel management doesn't work. Some surgeons extrapolate from hisbone disease total colonic ganglionnosis, where you can do or familial polyposis or ulcerative colitis where you can do a total colectomy and take the small bowel connected to the anal canal. The difference is that those patients have normal sphincter and normal anal canal. In anorectal malformation, it is contraindicated to do a pull-through if the patient is incapable of forming solid stool. So patients with cloical atrophies have different degrees of shortness of colon that goes from zero colon to normal colon. So, we, the obligation of the surgeon when confronted with this defect as a newborn is to separate the GI tissue from the urinary tissue, incorporate all the GI tissue in a single tube because these patients sometimes get separated pieces of colon, very abnormal blood supply, sometimes they have a pouch, incorporate everything in the GI to GI and open an end colostomy. The most common error in the neonatal period is for the surgeon to open an ileostomy and leave a piece of colon attached to the urinary tract. That piece of colon is not going to grow, it is not going to develop capacity to absorb water. It's going to absorb urine, it is going to provoke um um hyperchloremic acidosis that will interfere with the growth and developing of the patient. So the, in the neonatal period, remember separate the GI tissue from the, from the urinary tissue, open and end colostomy and then. Watch the baby to see how much that colon grows. If it is a short, even if it is a tiny piece of colon, it will grow and will develop more and more water absorption capacity. And then the question is sometimes they have a normal colon so they can have a pull-through and sometimes they have no colon, so there is no pull-through, but sometimes they have Pieces of colon and we are not sure as to how much, how much capacity the patient has to, to absorb, to absorb water and to form solid stool. If that's the case, we do something called bowel management through the stomach. You see, look at this, at this X-ray film shows a patient that was born with a small piece of colon, very abnormal. You see that that image is of a bear, is a pouch actually. With a a very abnormal blood supply, do not resect any piece of colon in patients. In dealing with anorectal malformations, every piece of of gastrointestinal tissue is extremely valuable because sometimes we use it for bladder reconstruction, for vaginal reconstruction, and because we need it for to to for water absorption. In cloical atrophies, do not let the urologist to use gastrointestinal tissue to make the reconstructions without you previously having decided whether the patient has enough GI tissue for a pull-through. That's the priority. So here you see a patient that was born with a very small piece of colon. But that piece of colon kept growing and now it becomes a sac. The question is, should we pull that colostomy down? If we are not sure, we implement what we call bowel management through the stomach. In other words, we kind of simulate that the stomach is the future anus, and we give the enemas through that stomach and the, the purpose of this is to see if we can keep the stomach back completely clean for 24 hours. If we keep, if the patient is capable of staying 24 hours with that completely empty stomach bag because of the enema that we give once a day, that means that we can take that colostomy down as an anus and the patient and the family knows the kind of effort that will require for for him to stay completely clean. Thank you very much. Thank you.
