We're going to go to the first, uh, slide. Um, we are going to try very hard in the next hour and a half to cover these interesting and controversial topics. Doctor Penny and I selected these topics because I think these are the ones most controversial, the ones that people really want to discuss what to do. And that includes whether to do a colostomy or a primary repair in an anorectal malformation, uh, how to manage a perineal fistula, What to do about the problem of anorectal malformations with constipation, how to manage the newborn cloaca, and this concept of laparoscopy. So, let's just simply go to the first picture. And maybe I'll turn this over to, uh, uh, ALP. If ALP has a comment. This is a, these are two newborns with similar malformations, and I'd like to know how you would manage this in Cape Town. Well, good afternoon. Good afternoon, uh, everybody, and, uh, thank you very much for inviting us to uh join you today and uh I wish you success with the, um, workshop, online workshop. Um, this is obviously, um, on the left-hand side, we're seeing a little skin bridge, a bucket handle and a rectal malformation. And on the right-hand side, uh, we're seeing a little median rahae um filled with white, uh, um, meconium-like substance coming through. So these are, um, low malformations and, um, our approach to, uh, this type of malformations would be to do a local procedure, perennial procedure, and oplasty. Um, type procedure, um, to, uh, to help with the decompression of the bowel as well as do the primary surgery, um, at birth. Mark, over to you again. Let me ask you, uh, what exactly would happen if this patient was very ill or very premature and you didn't feel comfortable taking them to the operating room for a, uh, a newborn repair? Let me see if, uh, if Marcella wants to give a comment on such a patient. Mar Marcella, are you there? I can hear you. So, Marcella, what would you do in this patient if they were quite ill? Let's say they had a coarctation of the aorta or very premature and you did not want to take them to the operating room to do a primary repair. What would you do? Uh, I think that, hello. Nice to see you. I'm happy to be here with you. And I have a group of people from the hospital, uh, and visiting surgeons also. And I think that still, the first picture is a baggage handle and it's very related to a, to a low type of anorectal anomaly and, and I would still go and do a, a very restrictivenoplasty. And the second one, we have had both cases. If the fistula is too long, we had patients in which we had put a colostomy on those patients, but still, uh, If the patient is OK, I would do a nanoplasty and, and just resect the, the skin down fistula. Uh, I have had only one case in the 2nd patient in which we found a second fistula. There was a perineal fistula and there was a little connection to the very low urethra also. So, only in, in, in that case. Can I, can I ask a question for all those who are watching? When you are going to operate this, uh, malformations, which happens to be the most benign of all defects in the spectrum of anorectal malformations, what is the most feared and common. Intraoperative incident that you must be aware of. This is very important because the problem is that we surgeons set up our minds to do a difficult operation and we get prepared for that, but sometimes we believe that we are going to do a very simple case and sometimes we have the capacity to make a, a big complicated case out of a simple one. So I'm going to repeat the question. Which one, which is the most feared and, um, most, most serious complications that may occur in the repair of these malformation? Anybody who wants to answer. Let's ask Yama. Yama, how are you? Very well. OK with you? Thank you. Great to see you. Um, you want to take Doctor Pena's question? Yeah, you mean, uh, serious complication for these babies or, Yes. Intraoperative. Intraoperative complication for this baby. Uh, for those patients, I mean, uh, low type, uh, male and rectal malformations. Yes. I think a lot of time malformation, you know, can be uh. Uh, You know, Rare, but, uh, the patient may have, uh, uh, renal disease and, uh, Uh, genital urinary problems, I think, uh, that is one of the You know we have to be careful about that, but otherwise. I, I don't think, uh, except the high tide, I think, uh. You know, I don't find out any reason. To fear. Abid Kazi says urethral injury, as well as the anomaly being higher than what was anticipated. Yeah, I, so I like that answer a lot. We have to be very nervous about the urethra, even when we're doing a relatively simple operation. Um, perhaps you could comment on, I, I often hear people are concerned that they don't believe the rectum is so close, they're afraid that the rectum is actually higher and there's a long fistula. Maybe you can, Make them less nervous about that, that issue. I think the point being is that the, is the rectum reachable here in both of these malformations? Yeah. When, When you see a perineum, like the one that you are looking in the, in your, into your screen, the, um, um, you can be, um, fairly confident that you are going to find the rectum in a low location, but occasionally, because what medicine and surgery is all about is exceptions in the, you're accustomed to deal with certain anatomic pattern. And then tomorrow you find a variant, a, a, a different anatomy. So occasionally you follow that narrow track and you, and you don't find the rectum and you can, you keep going up and up and up. And finally you find the rectum located much higher than what you thought. And at that point, you have to use your clinical judgment and your experience and decide whether to continue the operation or to go for a colostomy. But most of the times when you find a perineal fistula, you, most of the time you will find that, you can bet that the rectum is going to be located low, but as, uh, I think Marcella mentioned, sometimes you find the rectum high. Be prepared for, for the anatomic variant. It's the same as when you do a cholecystectomy laparoscopic, you are very happy, but the one day you find different anatomy and what, what experience is all about is to be prepared to deal with those variants. But, let's, uh, let's switch gears a little bit to, um, these two patients. And ask, um, uh, is surgery appropriate for these patients and what would be the indications you would discuss with the family. Maybe Sabine, could you take this one? Yeah, uh, hello everybody. I'm very happy to be there. Um, can you hear me? Yes, perfectly. So, um, on the left it's a girl on the right I think it's a boy. Uh, in the left case, uh, this is an anterior fistula, but, um, it's a lower form, I think. But um the perineum is uh is not great. It is inflamed, um, so I will propose firstly a colostomy uh to put the perineum um quiet uh in order to to make a good reparation. Uh, Doctor, uh, Langer, I see, has written a comment. He'll be one of our panelists coming up, but maybe I'd like you, Doctor Penny, to comment on his point that, um, uh, whether or not the fistula is opening anteriorly and if it's just a stenosis, that dilation might be a definitive operation. Yeah, well, uh, I was reading that, uh, that comment of, uh, of, uh, Jacob. Welcome, welcome to the, to the discussion. The imperial fistula, by definition, the, the anal orifice is located anterior to the center. Perhaps what you are referring to is another malformation that is, uh, pure stenosis. When that happens, most of the time, stenosis is a little deeper, not, not quite at the skin level. And, and that's a very interesting, uh, malformation. We have patients that were subjected to dilatations, and we just, it, it can be done, except that once in a while, you may find that the patient actually has a presacral mass, and, uh, that's why all these patients with perineal fistulas must have, And AP film of the sacrum, not only lateral. The AP film, because, uh, the, the most common malformation associated to a presaccrum mass is this, this group of perineal fistula. So, in the AP film, you see a defect in the sacrum and the, the, the presaccrum mass, the rectum and near the anus and interfere with the dilatation because the patients have, um, a very narrow fibrotic, uh, anus. And we have patients that have been subjected to dilatations that failed only because they missed the diagnosis of presacral mass. And another very important thing, the, um, The, this is the, the malformation that runs more frequently in families. In other words, when you, when we see a patient with a presacred mat, a defect in the sacrum, we screen all the members of the family for a sacral defect. I remember vividly a case with a perineal fistula in a female that I told the family my usual speech and said, congratulations, this is a good malformation. 100% of the patients operated by us with this defective bowel control, so your baby is going to be very, very good. And then I went to see the x-ray of the sacrum. The patient had a defect in the sacrum and a precircular mass, and I had to, I had to, To apologize with the family and tell them that unfortunately the prognosis is not the same and I have to say like an American politician that say I should say very few words because frequently I have to eat them. I think we have, uh, Paula now with audio. Uh, Paula, can you tell me what you feel are the indications for surgery in the female in this picture? Uh, OK. This has been, uh, a debate among us, uh, as, as a first, uh, watching, uh, to us if the, um, anus is adequately, uh, bigger, uh, like, uh, a 12 dilate, uh, 12 hagar, I wouldn't touch. And we've been following these patients. We have, uh, 21 girls now, uh, with a median age of 7 years, um, that has a slightly mislocated anus like this one and, and period with a, with a normal anal opening, not, not narrow as in in the male, uh, acceptable and wide anal opening, and so far we haven't touched any of them. One girl wanted to have a second opinion and they operated her and she's doing. Uh, well, anyway, so I, I can tell you that, you know, in, in my view, the reason to fix this female is that the hole is too small. The, uh, distal aspect is not good mucosa lined tissue. It's more fistula tissue, and it will remain stenotic and allow for a dilated rectosigmoid above and severe constipation. Yes. And also, you would like the hole to be perfectly centered within the sphincter. They will have good bowel control if you do not touch them, but as you know, uh, Paula, uh, and I have seen a number of patients that their bowel control is, is not absolutely perfect when they are adults. They have problems when the stool is loose or when they are having athletic activity. So I think the, the goals are, uh, to make an adequately sized hole, one that is in the center of the sphincter, and also the additional benefit is to give them an adequate length perineal body. So, given those details, what I want to respond to is, um, Luis, Luis's, um, question from Mexico, who's on the upcoming panel as well, um, asked, many surgeons are still doing a cutback procedure. So, perhaps, uh, uh, Doctor Pena can talk about whether he thinks that's a good idea or not. Well, the, I always like to talk about the, the man and his circumstances. Depends on the circumstances that you're working in. Let's, uh. Let's suppose that you are dealing with a 1 kg premature that has a perineal fistula with a very narrow anal opening and it's, uh, insufficient to empty the colon, so the baby is distended his symptoms. And in addition, the baby has a cardiac condition and is very sick, and you don't have a good anesthesia to take care of this baby, that's when, Conceivable you could do a good back, which is a, it's, it's an operation that we, we don't do, actually, because we don't work under those circumstances, and, uh, that doesn't hurt much the patient because it's basically a cut in the midline. So it's an ugly operation, either for a bad, it's, it's a, it's a good operation for a bad surgeon or a surgeon working under very difficult circumstances with a very sick baby. It's a temporary procedure. Uh, but it's very interesting that those babies subjected to that procedure also will have bowel control. So, um, the most important aspects of this malformation is to remember that it's frequently associated to a presacral mass and be aware of that, because people don't know much about that. And most important, most important is to, To tell the family that the good news is that it is the most benign of all defects, but the bad news, this is very important, is that the patient is going to suffer the worst constipation that you have seen in the spectrum of anorectal malformations, because for some mysterious reason, the lower the malformation, the more, the more severe the constipation, The higher the malformation, less constipation for these patients, with exceptions, of course. But the, the main problem in, in this kind of patient is constipation that is going to be there for life and requires a very aggressive management with an amount of laxative that is frequently 235 or 10 times more than what the book says, so we don't go by the book. Uh, uh, uh, has written a note, uh, here, um, how many would take the patient to the operating room and do electrical stimulation before deciding what to do? Um, does anyone have an opinion about that? Perhaps, uh, uh, Professor Liam from Hanoi, do you want to tell us if that's a good idea? Do we have you on the line? I'm sorry, I, I see you, but I cannot hear you. Uh, Long Lee, are you here? Yes, uh, uh, yes, from the left, uh, uh, figure you can see the girl suffer from a low type of imperfect annals. From our experience, uh, we start the treatment by annual dilatation, uh, for one month, uh, to. To prevent the dilatation of the rectal sigmoid, one month later we are going to perform the limited posterior sagittal approach for the girl. For the boy on the right, uh, side, uh, we, uh, in China, the surgeon, um, uh. Prefer to perform the cutback procedure because uh the functional result is good and it's also cutback procedure is also very easy to manipulate. I think it's important also to recognize most patients, it's very obvious where the sphincter is and where the anal opening is. I'll show you here. If this pointer to drag it on works. On this picture, you can see very nicely the ellipse of red, pink tissue. That's the center of the sphincter here. That hole is too small and too far anterior and not in the center of the sphincter. In the female, this is the sphincter. Here, that hole is just at the anterior aspect of the sphincter, but it is not in the center of the sphincter. All right, I, Mark, let me, Steve, uh, Steve, can we put a poll up to the audience? I'm curious who would perform a cutback anoplasty, uh, in, in, how do you want to phrase the question? Well, let's put it this way, who would simply open this hole and make it bigger? A, and who would mobilize this as a formal mini, uh, PSA. So, just to make it easier for Steve, so one is make hole bigger. Two is, is mobilize this, put it in the center of the sphincter and close the perineal body better. So mobilize and close the, just try to mobilize, and what's two? What's three? That's it? That's what you want? Three is dilate only. Dilate only. So we're going to, we're going to hear what that poll shows. Let's, let's go through a couple of quick slides now. Um, colostomy or primary repair for this male? Let's perhaps hear from, uh, Ivo from the Netherlands. Colostomy or primary repair. Hello, Mark. This is Ivo. Thank you from the Netherlands. First, I'd like to say we will probably win the World Cup soccer. Yeah, but this would also be, uh, a colostomy. Um, we generally wait 24 hours, um, Now there's something in my screen, but and we look at the bottom, whether it's flat or not, or what it looks like, and then you can fairly estimate whether it's uh uh bulbar or a higher fistula. This one would be probably a higher type of indirect malformation than we do ay. So, um, in the meantime, I don't mean to confuse you. You're voting on the female, uh, but we're looking at this male and Evo suggests doing a colostomy. Is anyone, does anyone on the panel advocate a primary repair of this male? No. Let's say we are at 24 hours of life. The abdomen is a little bit distended, and there is absolutely no hint of a perineal fistula. Is any, would anyone on the panel like to suggest a primary exploration for the rectum? No, no. OK. Um, what about this case? Mark, Mark. Yes, Evo. There have been some publications of, uh, of studies of people who do it, who do a primary repair, who say it's, Uh, it's possible, but, uh, I don't know what the panel thinks about it if the what the, what the dangers are, but I always, I'm always a bit, uh, afraid. Uh, uh, let, let me answer, answer to that evil. The, yes, of course, this has been a subject since 1948. There have been surgeons operating primary or non rectum malfirmations. Now, if, if you are lucky and you find the rectum right there, you can have a successful operation. If you have a lot of experience and you have done, it's conceivable to do it. The question is whether we should generalize the idea that, saying that it's a good idea to approach these patients primarily. So this is the type of case in which the so called invertogram that we don't use, now we use a cross stable lateral film. We put the baby in the same position that we do for posterior sagittal and take an x-ray film, placing the film on lateral side of the baby and the, the beam of the x-ray entering the other side, and you have exactly the same image of the so called invertogram. And let's suppose that in a case like this, you see the gas coming below the coccyx. If, if you see the gas coming below the coccyx, you can be sure that when you open posterior sagittal, you are going to find the rectum, and if you have experience, and if you are a delicate surgeon, a meticulous surgeon, you will be able to repair the mass formation, Successfully. So otherwise would be what we call an adventure, an adventure that would be, may become a misadventure, a misadventure that will have serious consequences for the babies. And we have seen that because now the, on the other hand, if you, if you try to repair these malformations primarily, And you don't find the rectum, you, you, the baby is going to have problems. And, and let me mention something. Jacob said that why is the perineal body important in females in, uh, because why not just to make the orifice larger in a perineal fistula? And the answer, the answer is I have seen adult patients that were never operated from this, and they have almost a normal, happy life, some of them, but I have seen others that seem to be very upset. Number one, because they don't like the idea of having the anal opening very close to the vagina for psychological reasons and, and, and reasons that in the past people did not confess, but now they like to talk about that, discuss that, and they don't like that. And second, Then there is a potential risk of serious injury to the rectum if the lady is going to deliver a baby vaginally. So those are potential problems that you don't see in the baby. The baby is not going to complain, but they, teenagers may complain and adult ladies may have problems. Jack, you're always so good at, at, we love you when you come on, because you always give great discussion. Call in. Let's, let's hear what you have to say, so if you can, uh, You have a lot of points to make, so don't hesitate for anyone to call in and make your points on the air. Let's go with, uh, this case, colostomy or primary repair. We're going to put a pole up. And then, uh, perhaps, uh, Alp, you can comment on what are, what is the unique features to this perineum that strike you. This is obviously a male baby with fairly flat buttocks, uh, and, uh, I can't see any signs of a fistula being present. So from, from the clinical, uh, um, information that we have and presuming that this baby is about 24 hours old, um, a newborn baby, um, this is, uh, looking like a high malformation. So, uh, we're very much on the safe side. We don't like the adventures, so we would, uh, recommend that a colostomy be performed on this baby as an immediate, uh, first, uh, procedure in a newborn in the 1st 24 hours. Yeah, it looks like, uh, it looks like everyone agrees with you. Yes, yes. How about this one?
