Decision making in Pediatric Colorectal Surgery - A webinar of case discussions

So, welcome everybody to the um webinar, Decision Making in Pediatric Colorectal Surgery. Um, I have the privilege to moderate this collaboration of the um UUSA Children's National in Washington, and the European Journal of Pediatric Surgery reports. We are also happy to have uh Mark Levitt. He is Chief of Division of Colorectal and Pelvic Reconstructive Surgery at the Children's National Hospital in Washington DC. And on top, we have an expert panel that you will see later. Nature and during the session of colorectal surgeons, international colorectal surgeons, um, Julia Brizigheli, uh, from, um, Johannesburg, um, Paula Mitria from Treviso, Alejandra Villanova from Madrid. Uh, Pim Slots from Rotterdam, Carlos Reck from Vienna, and, um, our, yeah, how do you say, the heart, the, uh, of everything, Gaia Tamaro from the um UUSA office. So Mark, um, it's a big honor um to have you here, here today and that you share your knowledge and expertise in decision making in colorectal surgery. How are you today? I'm great, Martin. It's so nice to see you and I look forward to being together again when the world returns to normal. Great. Um, we changed gears to a clinical picture. Patient two, if you see this patient in the, it's obviously an older patient in the, uh, outpatient clinic. What do you think? Like, is this a normal anatomy to you, or are there any thoughts of what is not right here? All right, Carlos, are, are you finished saving the life of the child that you were on the phone with? Um, so what we see here is we see a female genitalia, and if we go by the rule of counting how many holes we find, we find two holes, one which appears to be, uh, um, coming from behind to be, a, a pretty normal anus with, uh, what seems to be some, uh, lying on the sphincter. And in the vagina we have only one hole. Now it looks like a quite big hole. We have two possibilities here one for this to be a UG sinus or one to be a vaginal atricia. Um, behind the, the, the hole we see like some pits. From what I can see in this picture. But uh they are not deep enough to suggest uh specifically any of the two. So the way to go for this would be to do cystoscopy uh as a diagnosis. OK. Paula, do you have any opinion of or do you agree with that? Yes, there are only 2 orifices. So, the first thing, uh, you need to, to do some diagnostic workup. So, cystoscopy here would be, cystovaginoscopy would be uh the first, um, The first, uh, step to have an idea and then decide. It, it, this is not an emergent case as long as her kidneys are, are OK and the bowel is not distended because she has a, a normal anus and rather a normal exiting for the urine. All right, so is there anyone on the panel that thinks this is normal? No, no, OK. There are, it seems there are two holes. And there are two possibilities that Carlos described. And those need to be confirmed by cystoscopy. Now let me ask one quick question maybe for Thomas is why is whether or not the anus is normal so important? I, I, I think that it's. Uh, well, I don't have a good answer, of course, it's very good if it's normal. If it's abnormal, I would think that affects the, the whole structure, and you could think about whether this is the. Anteriorized anus. I was looking at the synthesis and, and whether there is a distance between the two parts of the synthesis, but I don't have a really good answer. Yeah, so I, I, the, the, the thing I wanted to make the point of here is if the anus is normal, then you have to be worried about a urogenital sinus. With a potential endocrine, particularly adrenal problem, although of course this is in no way virealizing. However, if the anus is abnormal, or certainly if there is no anus at all, then you need to think about a cloaca, and I can tell you there are patients like this who have been determined to be DSD cases in the newborn period and have been left for some time without a gender assignment. Um, and in such a case, you need to be aware of that issue, and of course you need to check your, uh, check the electrolytes. So we uh need to scope such a patient and determine whether or not this is a urogenital sinus with a urethra and a vagina, or that's a urethra and there's no evidence of a vagina, and I would call that a distal vaginal atresia case. And then we have to say, well, what are the possibilities there? And anyone wanna comment on that if this is a in fact a distal vaginal atresia with a urethra and an anus that are OK. Paula, do you have any thoughts about that scenario? Uh, well, the, if it's a distal vaginal trisure, then the urethral orifice is quite wide. And Uh, I, I would decide after the cystoscopy because, uh, there is no worry, no, no hurry to make any surgery if the two organs that need to work are, uh, patent, I mean, in the urine, urinary tract and the, um, colorectal tract. So I would, I would keep the luxury of the time to, um, do the cystoscopy and MRI and everything and decide with no hurry. OK, so let's say we do the cystoscopy and you show that that is only the urethra entering a normal bladder neck and a normal bladder. OK, then I would leave the, the patient, uh, you assess, of course, the internal genitalia with an MRI and if it's a girl, I would leave her uh for puberty to be fixed. That if it is distal vaginal atresia, I completely agree there's no urgency to this at all. But then what are the possibilities for the gynecologic anatomy? You've decided, Paula, to wait. Until puberty Um, I actually might do a a diagnostic laparoscopy because I really am very curious and I know that MRI, prepubertal MRI is not useful, really. It's very difficult to see the structures because they're very small, and I would like to know which of the situations I find if again this is distal vaginal atresia. The most common setup is normal ovaries. Ovi uh, fallopian tubes and nothing else. No uterus and no vagina. That's the most common. The second most common scenario is normal ovaries, fallopian tubes, and an upper vagina that would not possibly reach the perineum but might fill with menstrual blood. And for such a patient, you need to think about whether you need to do a vaginal replacement. And the least common scenario is truly a distal vaginal atresia, and in some of those cases you can make that reach with a laparoscopic approach. I think Martin, you recently had a case like this, you, did you not? Yeah, we had a um a case with, well, we currently have a case with batted Beetle syndrome. It's a vaginal atresia, looks like, almost like this patient and had a hydrocorpus that we drained with a a pig drain, pigtail drain via the abdominal wall. And uh now it didn't fill up again, this hydrocorpus, and now we are leaving it alone. Until puberty. That's, that's our plan. Yes, and then at, at puberty, I think you're going to plan a laparoscopic mobilization of the distal vagina, correct? Well, you know, our protocol is that we, we leave it up to the patient and we have this Visighetti method that they, they press little dimples in the perineum and create their own vagina and discuss it with the patient. So I'm not really sure what we're going to do, but this is certainly an option. But, but in your case, there is a blind ending vagina that's going to fill with menstrual blood. Don't you want to bring that to the perineal surface? Yeah, right, but what is the, well, don't you think, well, you could do that, but don't you think it would be nice to have some dimples, some introitous buildup in the perineum first to connect that distal vagina to? What commonly is the case is there There is no upper vagina to mobilize and you just need to uh dilate and then you can have a a usable vagina once the patient is older and ready for uh intercourse. But if there is an upper vagina, it has to have a way to get out. And I think the fact that you had to drain the hydroculpos in the newborn period means that there will be a vagina there. And a really nice option is to mobilize the distal vagina laparoscopically and then sew it to the perineum and you get a very nice functional and cosmetic result. And the most important question is how many holes do you see? In this case in which two holes are seen and one as most. Slightly a normal anus, then we need to determine if the anterior hole is either a vagina or a urethra. So the two options in this case is that it's either a eurogenital sinus or a vaginal atresia according to what the anterior hole is. So to determine that, you can do an EUA and a cystoscopy. And the reason why it's so important to define that the anus is normal is that um if you have a normal anus, then you need to suspect that it's most likely a eurogenital sinus and then you need to look and investigate adrenal problems by checking the electrolytes mainly. If the Nus is abnormal, then you need to suspect most likely the presence of ECOECA. So, uh, if the cystoscopy confirms, like in this case that the anterior hole is a urethra, then you're dealing with the vaginal atresia. And then it's important to determine the length of that atrisia and to determine the anatomy of the internal genitalia. Then, um, It's, um, as, as Mark was saying, the three most typical pictures of this, uh, from the inside is that the most uh frequent one is that you have normal ovaries and normal fallopian tubes, but absent uterus and absent vagina. The second option is you can have normal ovaries and fallopian tubes with the uterus and only the upper third of the vagina. And the third option, which is the most uncommon, is a true distal vaginal atresia. So in terms of consensus for operation, like it's not very easy to determine what is the right time to do an operation. In general, if there's no hydrocorpus, if there's no um uh then risk for the urinary tract, you can wait a bit longer until the patient is pre-pubertal. But if, so if, as a general rule, as Carlette was suggesting, if there's no uterus, then there is no rush because there will be most likely no blood and so no risk for the patient. If there is a uterus and there is a vagina, but it's retic, then initially you need to think of a vaginostomy and then you're not in a rush, but you should consider a pull-through or an interposition sooner rather than later. This is my summary. So that's a beautiful summary. The only thing I would just Add is that very often you don't need to do a vaginostomy. Not infrequently you can do intermittent catheterization of the common channel, but if you're going to do that, you need to prove that your catheter is going where you would like it to go and it's going into the hydroculus. It turns out that many of these patients, the urethra takes a very sharp turn anteriorly. So the catheter is preferentially gonna go into the hydroculpos and if you can successfully prove by ultrasound that you're doing the right thing and you're decompressing the hydroculpos and the hydronephrosis is improving, then you don't need to do a formal tube vaginostomy. So, um, Martin, I think it's. Sorry, can I ask a question for the summary? So that's true if it's a eurogenital sinus, but if it's a true vaginal atresia, then there will be no opening in the eurogenital sinus. right, of course, yes. So if, uh, if there is a true distal vaginal atresia, then of course intermittent catheterization will not work. You have to relieve the hydronephrosis by draining the hydrocopos. Uh, interventional radiology or laparoscopy would be the way to go, and Martin just did that in a case we discussed, and I would also recommend a pigtail catheter like you used, Martin, because as the hydroculus recedes, it's going to pull away from the abdominal wall and the tube can fall out, but a pigtail won't, won't fall out. Um, and by the way, before you remove the tube, it's a great opportunity to do a contrast, uh, contrast study and see exactly what you're dealing with. This is again only in the case of a hydrocpos.