Colorectal Quiz episode 12 Newborn ARM Part 1

Hey there listeners, this is Rod Gerardo and, Amanda Jensen from Cincinnati Children's. Have you downloaded the new version of the State Current Pediatric Surgery app? It's in the Apple App Store. It's in the Google Play store. You definitely want it for this episode of the Colorectal quiz because we have so many associated images that you're going to want to look at while Dr. Levit and Dr. Fischer are talking about it. So download it today, but until then, enjoy the episode. Today we are going to talk about anorectal malformations or imperforate anus. Amanda, how common is that? This is a congenital defect and it occurs in 1 and 5,000 live births. Okay, so what is it? It occurs when the anus, rectum, and nerves do not develop properly during fetal growth. And there's so many different anomalies that can pop up. Here's a few. It can be too small, it can be in the incorrect location. It can be absent or it can be entering into other pelvic structures such as the urethra or the vagina. Awesome. So, let's get into it. Without further ado, this is the Colorectal quiz. There is a reference to a baby born with no anal opening and what to do about it from 2,000 years ago. That is Dr. Mark Levit giving a history lesson about the Talmud, a specific reference to what to do when a baby is born without an anus. Then what should be done is you need to rub the area where the anus ought to be with oil and place the baby in the sun and then scratch it with a barley grain. That's probably the first medical description of an anoplasty, but I don't think we need a study to know it's probably not that effective. And I suspect all other patients who didn't have any way of getting the meconium out did not survive until more sophisticated surgical techniques came along. But we're not here for a history lesson. So Dr. Fischer, what are we going to talk about today? Anorectal malformations in the newborn. And I love it because this episode is not just for the pediatric surgeon. This is for a broad audience. This is really relevant for neonatologists, pediatricians doing newborn exams. Of course, gastroenterologists. Don't forget the people like me, the general surgery residents, surgery residents who are interested in pediatric surgery. All of them need to know how to do a good newborn exam. The good news is more than 90% of these patients, their actual anatomy can be ascertained on physical exam alone. So that's what we're going to focus on. All right, so let's go ahead and get started. If you're listening to this in anything other than the State Current Pediatric Surgery app, that's okay, but if you're in the app, go ahead and scroll down under the media player. Look at the first image. Here's what they have to say about it. All right, so let's talk about, let's start with the mail. What do you, what say you? What do you, uh, what do you see in these, uh, pictures? Obviously, when you meet a newborn for the first time, you have to take everything into account. Focusing on the colorectal part and pelvic issues, uh, the baby and picture on the left demonstrates what I believe to be is a boy passing meconium through the urethra and out the meatus. Look, I'm not the best general surgery resident, but even I know that means there's a connection between the GI tract and the urological tract somewhere. We know that the when you take boys and anorectal malformations and you try to categorize it, you could go from the most distal malformation being like a perineal fistula, and then when you start attaching or connecting to the urogenital tract, you have connections to the urethra or to the bladder. Okay, so we have to figure out what kind of connection this is. These are usually categorized by the location on the urethra. So is it rectoural bulbar fistula, rectoural prostatic fistula, or rectal bladder neck fistula? If you're having difficulty visualizing exactly what Amanda is talking about, you need look no further than August Rodan's sculpture of St. John the Baptist. The right arm is popularly used to describe the location of rectoural fistulas. Seriously, I'll put a link to the JPS article below. You know, you know that 95% of boys have a fistula somewhere. The the concept of imperforate anus is actually a bad term because they're all perforate. They just don't perforate into where they ought to be perforating and the vast majority enter into the urinary tract. Okay, how about the second picture there? The second panel, so is a picture of a boy, a newborn boy, but you see a sort of flat bottom, maybe a sacral dimple, um, a little hard to tell, it's not at a great angle, but looks like there's a little dimple, but no opening and no signs of meconium that I could see. Yeah, I agree. So you're sort of waiting in this case, maybe the baby will pee some meconium. It's nice to put a little gauze to check the urine for particulate matter. Um, I agree that bottom looks quite flat. I don't see any obvious perineal fistula coming anytime soon. This is probably a baby with a rectoural fistula that has not yet declared itself. How about the treatment for these two? Briefly. Um, I think both these kiddos are going to need colostomies and then ultimately distal colostograms and then a definitive surgery, which will be a subject of another another podcast. As stated, the presence of a flat bottom, meaning absence of a normal midline groove is usually associated with a very high located rectum and usually associated with a bad prognosis. All right, let's go to the second set of patient images. Again, if you're in the app, scroll down under the media player, there's a whole bunch of pictures there. Go to that second group of pictures. Uh, if you're at the Art Museum and you see this photo up on the wall, what does it say to you? Really questioning what kind of art museum Dr. Levit goes to, but here's Dr. Fischer's response. Looks to be a clamp taking a bridge of skin where the anal areas appears it should be or close to that area. This is a classic picture of a bucket handle malformation. Okay, so admittedly, this is really difficult to describe over audio. So please download the State Current Pediatric Surgery app. But here's my shot. So think of a bucket handle. That handle portion is the skin bridge. It's going basically from this patient's anus to their perineum along the midline and there's a clamp under into that that that space. Bucket handle, you can be very confident that you have a perineal fistula. This is really important because you might have a baby that's too ill to go to the operating room and you can be confident that right under that little bucket handle will be the perineal fistula and you can gently dilate that to get meconium out and spare that baby a trip to the OR. If let's say they have a coartation or something like that that you don't want them going to an OR quite yet and then you can do the definitive repair some other time. Um, the perinealal in this case is obviously anterior to the sphincter center, which you can see by a pinkish ellipse. The picture on the right, a great picture and we see this often as well. This shows a newborn baby male, a what appears to be a somewhat formed area that you could make out would be the anal sphincter complex area. Um, but I'm sure, I'm guessing that there is no hole there, but you'd have to do a little bit more investigating. But within the scrotal rafe, you could see meconium traveling within the scrotal rafe, um, and there's one pearly bead, uh, sometimes you see multiple, which is a calcification of the meconium that is within the scrotal rafe. You can also be confident that this is a perineal fistula, but these are a little bit more tricky because that fistula parallels the urethra for a long distance. And when we get to the session on surgery, which is not today, um, it's very important to recognize that potential for urethral injury. And I don't think you have to go crazy finding that fistula. You just basically need to unroof that meconium and then find a healthy rectum and make an anoplasty. In images three and four, Dr. Fischer and Dr. Levit are describing two different perineal fistulas. The first one being a bucket handle and the second being called a black ribbon malformation. Amanda, you're on a roll. Keep going. In the male patients with uh perineal fistula, one may see meconium coming out. In such case, the diagnosis is easy. Um, the fistula is always located anterior to the center of the sphincter. Um, so when examining these patients, it's really important to look closely uh for this opening and no surgical repair should be done before the first 24 hours because a patient may pass meconium through the tiny orifice, um, and uh we should be able to identify their perineal fistula. So to summarize with a bucket handle uh malformation, usually one can find the fistula orifice underneath of that strip of skin that we call the bucket handle. Um, we basically went through um the scenarios in in males. There's another scenario and that is a no fistula, quite rare, only about 5% of cases and not surprising if you had a trisome 21 patient, but the no fistula defect is almost uniformly at the same level as a bulbar urethra. So in summary, today we talked about four different types of anorectal malformations in males. As you can tell, there is a lot of variety and that is why the physical exam is so important. Very well said Amanda. That concludes this week's episode of the Colorectal quiz. Dr. Levitt and Dr. Fisher didn't leave us with a joke. So as your host, I'm going to take a little creative liberty here and give you guys a language lesson. What's the word for constipation in German? Anyone? Far from pooping. And with that, I think I've been hanging out with them for too long. Until next week, I'm Rod Gerardo and, I'm Amanda Jensen at Cincinnati Children's. And remember, knowledge should be free.