"Ureteric abnormalities" by Dr Karen Milford, Paediatric Urologist, Johannesburg, South Africa



Join Dr. Karen Milford, a renowned pediatric urologist from Nelson Mandela Children's Hospital, for an insightful on-demand teaching session on the approach to ureter abnormalities. With vast experience and overseas training, Dr. Milford will cover the diagnosis, management and treatment options for various ureteric abnormalities, such as Duplex systems and mega ureters. Whether they are a part of your daily practice or you encounter them rarely, this session will empower you with critical knowledge on these conditions. The session also includes a brief touch on urethrocele, discussion on risk stratification, identification of indications for specific investigations and more. Enhance your understanding and ensure better patient outcomes by learning from one of the leading experts in pediatric urology.
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This is an invited talk on "Ureteric abnormalities" by Dr Karen Milford, Paediatric Urologist, Johannesburg, South Africa, as a part of the Zoom academic meeting of the Department of Paediatric Surgery in East London, South Africa. This video is for health care professionals ONLY and NOT for the general public.

Learning objectives

1. By the end of the teaching session, participants will be able to describe the common presentations in patients with ureteric abnormalities. 2. Participants will be able to list out the steps of a comprehensive assessment and workup for a patient with a suspected ureter anomaly. 3. Participants will be able to identify and differentiate the various types of ureteric abnormalities, such as megaureter, duplex systems, and urethrocele. 4. Participants will understand how to interpret key findings from diagnostic tests like ultrasound and voiding cystourethrogram (VCUG) for these conditions. 5. Participants will be aware of the management options, indications and contraindications, for the major types of ureteric abnormalities and apply it for treatment decisions.
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Computer generated transcript

The following transcript was generated automatically from the content and has not been checked or corrected manually.

Um Can you see my screen? I can see it. Ok. Uh We'll start in a second. Um Good afternoon, everyone. Um Welcome to the Zoom Academic Meeting of the Department of Pediatric Surgery. Um Today, Doctor Karen Milford is going to give us a talk on an approach to ureter abnormalities. Doctor Milford, um he is a pediatric surgeon and urologist at Nelson Mandela Children's Hospital in Johannesburg. Sorry, I'm not sure what has happened to Ken's photo. It was here, but the computer seems to have eaten it up. I'm sorry about that. Um And I think she is probably the only qualified pediatric urologist in the country. Um uh If I'm correct, she did her undergraduate training in the University of Pretoria. She did a pediatric surgical training at Red Cross Children's Hospital in Cape Town. And subsequently, she did a fellowship in pediatric urology at the Hospital for Sick Children, Toronto Canada for over 2.5 years. Currently, she is an honorary lecturer in the Department of pediatric Surgery at the University of Witwatersrand and consultant at the Nelson Mandela Children's Hospital. Um Right. Doctor Milford is going to talk about uh uretic abnormalities and without much uh introduction, I will request her to start her presentation. So currently you can share your screen and start your presentation. Thank you, Karen. You can share your screen and start your presentation please. Sorry, it keeps saying share screen has failed. I'm gonna try again. Right? Can you guys see that? Yeah, we can see it. You can carry on. Thank you. Um I'll briefly put my video on just to say hi. Um Yeah, so thank you for that nice introduction. Um I think uh just as a qualification, I think we do have um many um very experienced pediatric urologists in the country and I certainly have trained with some of them. Um I think maybe I'm just the only one at the moment who did a fellowship overseas. Although to be honest, we're not um really sure. Um So I'm just gonna do a talk today on an approach to ureteric abnormalities because um, it's actually something that is not um super common, you know, we read about it a lot but we perhaps don't actually see it in our practices. Um As much as what, you know, our, our teaching would train us to expect. Um, and I found even when I was in Toronto, um there's a lot of controversy about how to manage um, urine problems, um perhaps, um because there really are a lot of options and we're not always um sure what the best one is. So in this talk. I'm just gonna um touch on the presentation and investigation of patients with ureteric abnormalities. Uh We'll briefly look at the differential diagnosis and then I'm gonna look at the management options, chiefly of Duplex systems and um mega ureters. Um I'll just briefly touch on urethrocele, but in the end I decided not to um dedicate too many slides to these because um I think urethrocele and themselves are quite complex and um we could talk for quite a long time just to about the management of these. Um So moving on to presentation, I think the presentation of these anomalies is really dependent on your um uh clinical setting. So, um you know, in Toronto, I think the vast vast majority of these were picked up antenatally. So these um these um infants will have antenatal hydronephrosis on their ultrasounds. Um Some of them may be picked up incidentally um in patients undergoing um imaging for other reasons um such as suspected appendicitis or, you know, um yeah, nonspecific abdominal pain. Um And then I think a, a large number of our patients present with symptoms. So um uti or infection of um the obstructed system or the refluxing system um is common. Um some of them will present with pain, um particularly the older Children. And I think um those patients with um primary mega ureters um sometimes get stones and um they can, they can present with pain. Um urinary incontinence um is an issue particularly in girls with ectopic ureters implanting um beyond the sphincter complexes. And then um we also have a subgroup of patients who present with bladder athletes obstruction due to obstructed urethrocele. Um sorry, due to prolapse urethrocele, um which is something I think um pediatric surgeons and urologists should know about to know how to manage um urgently. So, just on the workup, um as always we start with a history and exam. Um So yeah, you want quite a clear history of urinary tract infections. And I think um it's always good for your practice to um to know what the definition of a uti is. Um because patients will be investigated or treated for UTI S without definitive evidence or without really meeting the criteria. Um They may have um underlying pathology even um if they didn't have a true uti. Um but it's important when you're deciding on your management, going forward to know whether or not your patient and um is having tr UTIs, um a history of trauma, particularly in older Children is um important to know um particularly whether or not there's been any perennial or kind of like bicycle or sort of what I call pre branch um trauma. So, like falling onto something that may have caused um a urethral stricture um always good to inquire about antenatal scans. They may have been in a history of mild or moderate hydronephrosis that um maybe disappeared with time, but it's also useful to know if the antenatal scans were reported as being completely normal. Um You're obviously gonna look for palpable masses. The genitalia are important to in inspect um particularly um in girls where you're kind of looking for evidence of a prolapsed um urethrocele. And then um with all urology patients, always, always, always, it's important to inspect the lower back and really satisfy yourself that there is no evidence of um an occult spinal abnormality. So, um when you're looking at a patient who's either as always presented with a uti or who has a history of antenatal hydronephrosis. Um You're gonna start with ultrasound because it's the least invasive and the um the easiest to start with. Um and the first thing you want to know is about the kidney morphology. Um So specifically, um what the, you know how much hydronephrosis there is if there is hydronephrosis and also whether or not the parenchyma is affected. So, is there parenchymal thinning? Is there evidence of um parenchymal scarring? Um Yeah. Um and then also size of, of the kidney. So, is there evidence of dysplasia um or of a small kidney laterality is important. Um And it's also very important to know if you're dealing with bilateral or unilateral disease. Um We'll get into that at the moment and then an actual estimation of the size of the ureter is quite important because um the ureter really needs to be more than 0.7 millimeters in diameter to be truly classified as a Megaureter. Um uh And all of these things are also important. Um Seriously. So you want to know if your hydronephrosis is getting better or worse um as the same goes through your hydroureter. Um So if you're um some of these Children may or may not, and we'll just kind of talk briefly about like how you're gonna go on to investigate Children when we talk about risk stratification. Um But uh in Children who go on to have a VCG, um which obviously involves um catheterization, ionizing radiation and can also be in my experience a bit of a difficult investigation to do in older Children. Um So you really need to be clear on your indications for doing it. Um So the first thing is that um you're really going to want to do a BCI think in Children with um bilateral hydronephrosis or hydroureter. Um Children with, I would say with recurrent infections, um and Children with renal dysfunction because as always, what you're trying to do is exclude valves which can present with unilateral hydroureter nephrosis. Um and can often have actually a very subtle presentation in terms of upper tract findings, but really shouldn't be missed. Um The bladder is also important to look at. So you want to look at um evidence of um urethra Seles and then I think always get an idea of whether or not there's trabeculation. Um if there's um diverticula and other evidence of what I would think would be a neurogenic bladder. And then um you obviously want to know if there's reflux. So um functional scans once again um is as you're moving down your diagnostic pathway. Um And really when you are moving on to asking for a functional scan, once again, you're asking for very specific questions. So you want to know the differential function of all the renal units. So you don't just want to know the differential function, the differential between left and right. If you're dealing with a duplex system and you are going to subject your patient to um nuclear imaging, then the um the enologist needs to be able to tell you what the differential function is in the upper and lower moieties of duplex systems as well. Um And the reason for that we'll get into in a minute and then um if you're going to do a diuretic renogram at the same time, um because you're trying to, you're essentially trying to look for evidence of obstruction either um at the level of the U PJ. Um So the um ureteropelvic junction or at the level of the bladder. Um so, which would indicate more um uh urethro vesical junction obstruction. And then just briefly to mention that you will um seek other imaging if you're concerned about a a spinal lesion. Um and also in patients um where you have any reason to suspect that they will have um renal dysfunction. So that's actually the vast majority of the patients. Um but you don't want to miss a patient who um is already having um a sort of renal disease as a, as a result of their. Um yeah, the ureter abnormality. So, when we thinking about um a hydroureter um in the context with or without hydronephrosis, although most of them do, your differential is sort of your megaureter. Um and this can be subclass into your megaureter that reflux. So megaureter really just refers to um an enlarged dilated hydroureter. Basically. Um As I said before, technically, um it should be more than 0.7 millimeters, 00.7 centimeters um distally. So these can be due to reflux purely. Um So dilating reflux, um They can be due to a primary obstructed megaureter and I'll discuss that in a bit. They can be obstructed with reflux at the same time. And then you also get these um subcategories of ones that are neither refluxing nor obstructed. And often these um Children have um more of a systemic illness that might be inducing polyuria such as diabetes and syphilis. Um the duplex systems um are something to think about your radiologist really needs to know how to um look for a duplex system in the upper tract urethrocele. You will see hopefully on ultrasound. Uh it can be difficult and then, as I mentioned before, um I'm not gonna talk too much about secondary megaureter. But whenever you are dealing with a child with hydronephrosis and hydroureter, it is always really important to make sure that this isn't secondary to lower urinary tract pathology such as bowels, atresias stone disease, um or a neurogenic bladder. So, management goals as always in um urology are are to preserve renal function. And so to keep the kidneys healthy, to minimize infections and then to achieve continence. And when I think about the management strategy for a a hydroureter, um The first thing I try to do is decide how high risk my patient is. So, are they at risk in terms of losing renal functional renal units? Um are they at risk of developing an infection? Um and are they at risk of kind of deteriorating in many of these patients? It is possible to be conservative. Urological surgery can always be as simple as we think it should be. Um it tends to be highly complex, I think um and where possible we should be conservative and not do more harm. And then the right operation at the right time in the right patient, which I think can be quite difficult to decide on. So when you're dealing with a hydroureter, you really, you need to kind of stratify the risk. And there are various ways of doing this. There is IMA image based um risk Strat stratification, which is upper tract dilatation scoring, which is really just using ultrasound. And as I say, in urology, it's almost always divided into third. So you've got the low risk patients where, you know, you really don't need to do anything. And then you've got high risk patients where you know that there's probably a problem and that you probably are gonna have to have to do something and then there's medium risk group which is somewhere in the middle. So, um on the left of my screen here, we're just looking at ultrasound based criteria which look at the APD, which is a very objective way to look at hydronephrosis and has the benefit of being reproducible where ultrasound can often be quite variable. Um There's another grading system. So the SF U grading system which looks more at clubbing of the K CS and sort of a global picture. I haven't actually included a picture of it in this um presentation, but um I think it's a little perhaps gives you a bit more information but is much more subjective. So it isn't always um the best system to use for people who are not very familiar with pediatric renal ultrasound. So obviously, the um the worse your hydronephrosis is, the wider your APD is um the more parenchymal damage or abnormal parenchyma you have and the more abnormal your ureter is um the higher risk you would consider your patient. But I think the clinical picture is really, really important as well. So Children where there's a history of infection and particularly when there's a history of recurrent uti s could always consider high risk and consider intervening um Children with a history of bladder athlete obstruction. So, if their urethrocele has occluded, their bladder neck and they've had an episode of um being unable to void. Um I would consider that um an indication to intervene and then if there's an evidence, if there's evidence of renal dysfunction, so your parenchyma is getting thinner on your functional studies, you're seeing a wide differential um or you know, you're seeing deterioration in renal function on your um lab results, then I think you need to consider intervening, but we'll talk about intervention in specific settings in a minute. So I'm just gonna briefly touch on antibiotic prophylaxis. Um I think uh so there's quite a lot written about antibiotic prophylaxis um for various um upper tract abnormalities and looking at the potential for them to decrease um infections. And I think the the studies, most of us are mostly familiar with are the studies that kind of looked at um continuous antibiotic prophylaxis in Children with reflux. So many of these hydroureter patients do not have reflux, they've got an obstructive system. Um And I think it's kind of institution dependent, to be honest that the institutional culture often um determines how often antibiotic prophylaxis is used. Um But there is evidence that it shows it decreases the incidence of uti and obstructed um patients who are high risk. Um One third of primary obstructive megaureter patients require a single hospitalization for a uti and that includes those who have had some kind of decompressive procedure. Um And we um we do think that the antibiotic prophylaxis decreases the chance of that. Um And then, you know, there are a couple of guidelines and consensus documents that do recommend um prophylaxis for the 1st 6 to 12 months of life. Um and then stopping it after that and seeing how things are going. So just firstly, let's just talk about who we probably don't need to intervene on. Um And firstly, those are the the Children with um asymptomat incidental asymptomatic duplex systems. So those where the duplex system was um picked up antenatally um or picked up incidentally uh for an ultrasound for another reason. Um and those patients, um you know, you can have duplex systems that have absolutely no hydronephrosis at all and uh you wouldn't intervene there. Um Asymptomatic urethrocele are sometimes identified. Um They should be in a cycle, they shouldn't be causing any obstruction, the Children shouldn't be getting any infections, those can probably be left alone. And then the primary obstructive megaureter, the vast majority actually resolve. So like about 80 to 85% actually resolve with time. Um So those Children who have not had any infections who've got a, a ureter less than a centimeter wide have got a normal looking kidney. Um and their split renal function is not concerning um can um also be observed who need surgery. Incontinent girls, um are do need um to be addressed. Uh If the ectopic ureter is implanting outside of the, the sphincter complex, um, you won't be able to resolve the incontinence with anything other than surgery, we can talk about surgical operations in a minute. Um If there's progressive dilatation of either pole, um but probably in particular, the upper pole and then obviously in all three of these groups, we say it over and over again. But Children who are having infections need to be um addressed. Uh urethrocele, like we said, those who are prolapsing um once again, need to be um addressed um where we're seeing progressive dilatation, even if the urethra cle itself is quite small, but we're seeing progressive upper tract dilatation. Um uh intervention should be considered once again. Um if there's deteriorating renal function and then if the urethra CS are bilateral um primary obstruction, ne I actually discuss this again in more detail later. So I'll get into it there. So let's look briefly at Duplex systems and before we um go into kind of the management options, I'm going to just do like a quick overview of some things that are sort of important to understand. Um when you're thinking about your systems and from a laser pointer on. So the w got mayor rule is um important to remember because it determines so much of the pathology and um it guides so much of the management. And that is that the upper pole obstructs and um the upper pole is the ectopic ureter. Um The lower pole tends to reflux and that ureteric orifice is more orthotopic. Um Sorry, II, get rid of this pointer. Um Let me just go back. So this kind of guides our how people present. So, asymptomatic, as I said before, they can have a complete or an incomplete duplicated system, um they might be going into a single ureter. Um And these are often um an incidental pick up. But when you have a known duplex system who is presenting with UTI S, um it can be difficult to know what the source of the uti is because you have an obstructed upper pole. And we know that any obstruction within the urinary tract can predispose to sepsis due to stasis. But then you also have a reflux in lower pole and we know that reflux also predisposes to infections. Um Sometimes the ectopic ureter can implant in the seminal vesicles in boys. And these boys can present with um orchitis, which is why whenever I have a patient who presents with an episode of orchitis, um even if we haven't successfully grown an organism, I will proceed to a normal um uti workup and I'll do start with an ultrasound for them and proceed from there. And then um as we know, you can have um you know, upper poles or you can have upper moieties that are not obstructed and not dilated. Um but an ectopic ureter that um, implants below the sphincter complex and can um present with incontinence. Um later in life, these girls are often actually picked up quite late because it's quite a um you know, at around the age that they go out of, it can be quite a difficult history to get and some of them also do have an element of voiding dysfunction. Um So often those things are addressed first. So the constipation and um you know, generally these, these girls often present sort of without a uti and without any kind of red flags. And so it will be managed as um Children avoiding dysfunction first. And um an ultrasound is not necessarily part of their workup if there are no red flags. So it can take a while for their, their duplex system to be identified. Um But once it is, then um you can offer them some options. So, like I say, said, when you do have a problem, um usually an infection in a duplex system, you need to work out where it is. So the ultrasound starts off by telling you which moiety is dilated. Is it the upper moiety, the lower moiety or both? Um the VC? Um we'll look at some pictures now, it tells you if there's reflux and both the ultrasound and the BC can help you identify your ureter scale or, and then the functional scan. So um at the NSA or A mag three are important in that you want to know. Um Exactly how much function each moiety contributes to the, to the whole. And that is because if you've got an upper moiety which is contributing like less than 10% of function, um it affects what kind of operation you may choose to do for your patients. Sometimes it doesn't. Um But if you've got a renal, so an upper moiety um that has more than 20% of the, the global function, you would really want to try and preserve those renal units less than 10%. I think um many urologists would be kind of comfortable with sacrificing those renal units. And then we all kind of have difficulty with the units that are in the moieties that are contributing between 10 and 20% or I think most of us would probably um try to preserve them. Um Mr S are, I think actually to be honest, uh less useful than what we would like them to be. Um But they are kind of useful in determining the course of an ectopic ureter, especially in patients who don't have um dilatation or hydroureter. And you are really suspecting um in girls, incontinent girls um an ectopic ureter, um uh sometimes the ultrasound imaging can be quite, you know, not really super helpful. Um And then the Mr can give you an idea of whether or not you've got bilateral duplex systems or um unilateral. And um what the course of those ureters is, I've just included some um images here um of. Yeah, so this is just um kind of a duplex system to give you an idea. This is probably a normal functioning lower pole and this is a probably an um obstructed upper pole. You would also, I mean, this is just a static single image, but on your ultrasound, you're also gonna get an idea of whether or not that upper pole has got good parenchyma or not or if it's just kind of, you know, a cystic nonfunctional unit. And here we, you have a VC which is showing reflux. Um I have to say like, interestingly enough for me, like white got May, you know, it tells us that it is meant to be the lower pole that refluxes. But um we'll talk briefly about urethra C in a second. Um But, you know, it isn't always, you know, sometimes both um poles reflux. Um And that is kind of, I think useful to know um before you actually proceed to anything. And this is really, so when you are going to manage these patients surgically, you really need to have a clear idea in your head whether or not you're addressing, trying to address the obstruction, the reflux or both, um particularly when you consider that whatever you do um is not necessarily going to solve the problem. So you can do, you know, whatever procedure it is, they might still come back with a UTI and then you need to ask why that is so a common sheath reimplant. Um, I think is probably the most conventional, um, surgical procedure. Um, and the reason, um, it's favorable is that it addresses both issues in one go. So it's, um, pretty conventional. Um, it addresses the reflux in the lower pole and it addresses the obstruction in the upper pole. Um I'll have to say that, um, you know, as much as we teach this as like, you know, the kind of gold standard of the procedure. It's not actually a very easy procedure and I think, um, not a procedure that people who don't do a lot of urology and particularly upper tract work are comfortable with. So, um, you know, we talk about it often but, you know, the occasion to actually do an operation like this doesn't arise very often. And so I think a lot of people are not, you know, super comfortable actually doing it. If you've got a very dilated ureter, you need to consider tapering or even um excising part of the ureter, we'll talk about more about that when we discuss the primary obstructed mega ureters. And then also, I think, um most, you know, more and more urologists are quite reluctant to do, um, these sorts of reimplant in very small babies. So those are babies, I would say less than even nine months to a year old. Um because you do risk damaging the bladder and causing bladder dysfunction uh later on. So, urethrocele puncture puncture um is a, a pretty simple procedure, particularly for um Children who have a big urethrocele in an obstructive system. Um that may or may not be prolapsing but is also occupying a whole lot of bladder volume. The problem with um so urethrocele puncture is very satisfying in that your obstructed system gets um relieved, nice and quickly. Um There are case reports of like the urethrocele, like sort of resealing over and um recurrent obstruction. But for the most part, I think, um you generally often get like relief, relief of your obstruction. Um and time for the baby to grow. The problem with ureteral puncture is that it really aggravates your reflux. And when you've got a patient with a duplex system, I can tell you from personal experience is that there is nothing more frustrating than or like kind of worrying. Then knowing you've got a patient um with duplex systems where you puncture the ureter cele and now they're back with a repeat uti um you ultrasound them and you see the upper pole is a little bit decompressed. But at this point, if you do a VC, you're gonna see massive reflux now into that upper pole. Um And sometimes you even see contralateral reflux and um reflux into the lower pole and then you kind of really stuck um in general, we treat the uti and maybe put them on prophylaxis and hope that it gets better. Um, but some of your, I mean, I'm not gonna talk much about reflux today. You know, after you puncture the urethrocele, you can't really deflux that ureter. Um Yeah, if they come back with repeat infections, you don't know, you know which ureter of the three or four ureters that the patient has is causing the problem. Um And in these patients, if they have recurrent uti S, you would be obliged to do a common tre implant. So, uh ureteroureterostomy is actually um an operation that is quite nice. Um So what this does is it Revo resolves your um upper moiety obstruction without um causing without you needing to do a re reimplant at the level of the bladder. Um And um without, well, we'll talk about um ureter in a minute, but you don't need to resect any part of the kidney. Um It preserves the upper pole function and it's generally not actually a very, I have to say difficult operation. Um We would usually recommend doing a cystoscopy before and putting a double J stent into the, the orthotopic ureteric orifice so that you've got a, a stent in your um lower pole ureter so that you can feel it. Um You can do a little Gibson incision um which can be ultrasound based. You know, if you've got a di dilated ureter, you can identify the ureter sort of here at the edge of the bladder. Um And really, all you do is um anastomose this um upper pole ureter into the lower pole. Um ureter, um this can be a challenging anastomosis when there's a huge size discrepancy. So these upper pole ureter can really be very dilated, which can make this anastomosis difficult. And I think um it can also be a bit concerning because you don't want to. So what you're doing is taking generally this lower pole ureter maybe has a bit of reflux, but on the whole, it's quite a normal healthy ureter and you're now going to open it and plug an amal ureter onto it. And there is worry for anyone. I think that you're gonna damage this lower pole ureter and they knock off the whole kidney. Um But the advantages are that it does avoid difficult bladder surgery and can be done in quite small babies. Um And then once again, this doesn't um sort out your lower pole reflux. Um You know, if that is a concern for you, um I would say that if you're still getting recurrent, you know, as always likelihood of uti is there. But if you're getting recurrent UTI S and you've got proven ongoing reflux into the lower pole after this procedure, I think you would once again be um obliged to do a reimplant. Um the upper pole ureteronephrectomy. So this is really only indicated in Children where the upper pole is causing problems but with very little um yeah, with very little benefit. So if this is a pole that is perhaps very dilated, it might even be causing compression of the lower pole and mass effect on the lower pole once again, um recurrent uti s and also even girls who are perhaps um incontinent from their ectopic ureter. So this gives you definitive removal of the problematic pole. Um but this is not a super easy operation. Um It can be done. I think most people, a lot of urologists would elect to do this robotically or even laparoscopically now. Um But it can still be quite difficult and injuries to the vascular pedicle of the lower pole during this operation are actually quite well described. Um So you actually need to be pretty comfortable with the surgery. Um So, you know, a confident, um I think renal surgeon um know your anatomy really, really well. Um And then it's important that you really need to resect all of the upper pole parenchyma because um if you don't, uh the remaining parenchyma will continue to secrete a bit of urine. Um And you can get urinomas and other um uh irritating problems. Um So I'm gonna talk about because I was in Toronto and this procedure was described there. And um there's a lot of interest in it is the, the concept of ureteric clipping. Um So it's very, very controversial. Um I'll show you a screen grab of the article heading and if anybody's interested, I can send you the article on it as well. Um, but it's kind of controversial because that sort of goes against everything it taught in urology, which is that the urine must leave the body. Um, but, um, it's advantages are that it's very easy. It seems to be particularly useful in girls with dribbling. Um, the one I would say absolute contraindication to it is infected systems. So, um I would actually not attempt this pro procedure in a patient who has ever had an infection in the upper pulse system. Um because the complications that they described in those patients were fairly significant and what I will say as well is that the group doesn't actually, you know, so they haven't written up the long term results. Um These are fairly short to medium term results. Um And so we're not really sure actually like 1020 years down the line, what is happening with these patients, but just to um kind of run through, um maybe let me see if I can go back. So basically, um in Toronto, this is done as a, usually it's done as a laparoscopic procedure but can it can be done open as well. Um And what they do is they first do a cystoscopy and once again, put a stent, you can just see the, sorry, let me put my laser back on, you can just see the green of the stent here in the um the lower pole um orthotopic ureter. And um very simply what they actually do is just pop a couple of Liger clips onto the ectopic ureter. Um So yeah, it's one or two Lager cips and then they divide it. So this procedure I have to say is, yeah, very quick and uncomplicated can also be done through a very small and kind of lower quadrant incision. Um So the reason they felt confident um attempting this procedure is that firstly, the transplant that just tells us it's safe. So there's a subgroup of transplant patients where um for one reason or the other, the native ureter had to be sacrificed and um when the transplant kidney was put in. So I think some of these are patients who had um kind of ureter to ureter anastomosis because the donor ureter was perhaps not long enough or had been to devascularized to go onto the, the um to the bladder. Um And in transplant, rather than doing a complete nephroureterectomy, a lot of these ureters are just tied off and over time the the kidneys and he stops producing urine. Um and there won't be any complications. Um So this is obviously when you're clipping that um upper pole ureter, you're essentially defunctioning the upper pole because um it is obviously not able to evacuate its urine anymore. Um And so to my mind, this is an alternative therapy to an upper pole nephroureterectomy, if you have an upper pole, that is still providing a lot of function. So, on your functional scan, it's got um functional more than I would say. Um 10 15%. Then this is not, I don't think an appropriate operation for those patients. Um When they did the study, they divided their patients essentially into four groups. Um So there was the duplex of the ectopic ureter. These were the um the girls who had incontinence, a duplex of a large urethrocele. Um and then what they called other duplex and single system kidneys. Um So they did the operation, they noted that the incontinence resolved itself. Um They continued to monitor hydronephrosis and I have to say that um in all the, in all groups, the APD of the upper pole does actually increase. But when they did the statistical analysis, it was only significant in the girls with incontinence. So even though there was still some persistent hydronephrosis and perhaps even increased hydronephrosis, it wasn't statistically significant. The urethrocele I think um in particular, were very rewarding. So the size of the urethra celes um was significantly decreased after the ureter clipping. Um I saw some of these patients um long term and we can, perhaps if anybody's got questions about this procedure afterwards, we can talk about it. Um moving on to the primary obstructed megaureter, which I think can be um like a management challenge for um many people. So once again, we're gonna confirm the diagnosis and um stratify the risk and you really want to be um, have very specific questions for yourself and for your radiologist, um when you're doing this, this process because it really, really directs therapy. So sorry that it's kind of gone hazy on the side here. But the first thing is you really want to know about your renal parenchymal quality because once again, I said, um a large majority of these actually resolve spontaneously. Um So you really need to use your investigations and kind of a clinical history to decide where you're gonna intervene. So, parenchymal thinning um is not a great sign and um progressive hydronephrosis and worsening kind of parenchymal thinning um is an indication to intervene the ureteric diameter. So this is a patient with a very, very tortuous ureter. I think her diameter was kind of 1.5 centimeters at its maximum distally. Um the ureter diameter on its own is not kind of won't guide you particularly with regards to surgery because even very wide ureters can resolve on their own. But um you kind of wanna get serial measurements and if you've got a ureter that's increasing in size, you might want to think about intervening there because it's a megaureter, you really do want to look for reflux. Um You wanna confirm nor normal urethra, this is very important. You wanna make sure that this is a primary megaureter and it's not a secondary megaureter due to um valves or some other kind of lower urinary tract or bladder pathology like a spinal effect. And then the functional scan, once again, very specific questions, you want to know exactly how much is the difference between the two kidneys. Um And this is also important um from a serial point of view. And then I would usually ask for um a diuretic renogram at the same time um and ask for the technologist to look at both the kidney itself and the ureter um to see if they empty. Um This is obviously a very extreme picture but you do get um Children who have AU PJ obstruction. So you um they are U PJ, we all know what that is. Um But who also have an element of hydroureter. Um And then the question for yourself then is, are you gonna do a pil plasty or do you actually need to um assess the, you know, address the UVJ? Um And that's where um a nuclear medicine unit that can kind of separate these two organs, the kidney and the ureter for you and tell you um at what level the obstruction is, is very useful. Um So, spontaneous resolution is possible. And that's the thing because often these Children have a very traumatic and very sort of um terrifying ultrasound. But even so, um many of them get better and the theory behind this is that um the pathology is in the distal segment of the ureter which remains a little bit AAA dynamic, but it is perhaps the last part of the urinary tract to mature. And so um in the in in infancy, sometimes, you know, there is maturation here. So ureters that are less than 10 millimeters in diameter, the vast majority resolve. But even those that are more than 10 millimeters in diameter. A number of them will not resolve completely on ultrasound, but they will be asymptomatic. So they might not be having infections. And when you do your functional scan, you might see that there's good drainage and that there's good differential renal function. And on serial ultrasounds that the hydronephrosis doesn't progress or it even gets a little bit better, they might never have a completely normal ultrasound. But um functionally, things will be fine. And so even the very large ones, only about one in five will actually go on to need surgery. So who definitely needs surgery um As always for recurrent infection. So about one, like I said, about one third of these patients will definitely have a uti in their life. Um even if you operate them, um Children with progressive hydronephrosis on serial ultrasounds. And here is really a question is, you know, how often should you ultrasound them? Um If it's an antenatally diagnosed um hydronephrosis, you want to get an ultrasound, I would say within the first two weeks of life. And then um for me, if it's a significant hydroureter. So more than 0.7 millimeters in diameter if there's some um parenchymal thinning, but in the absence of a uti, so you've still got a, you know, an asymptomat symptomatic and healthy neonate. Um I would usually opt for a repeat ultrasound in 2 to 6 weeks. Um Just depending on how, yeah, I'm feeling. Um I think there are some other um experienced urology people on this call who can maybe tell me how they go about it. Um And there are some kind of clear um protocols that different institutions follow. Um But that would kind of be my strategy. Um parenchymal thinning, like I said, um particularly if there's progressive parenchymal thinning, um I kind of start to be a little bit worried about as I said, the ureters that are more than 10 millimeters and I'd probably observe these more closely. And then um I think this is where, you know, the functional nuclear medicine studies are really useful. So if your affected side has a different differential renal function of less than 40% or some books would even say if the difference is more than 10%. So if you've got 45% and 55% on one side, but also if on serial functional imaging, you've got a deterioration of more than 5% over time, you should consider intervening. Um This is over quite a long time because you're generally are doing mag three every kind of four weeks, you're probably spacing them out by 3 to 6 months. Um And usually, I think if people are that worried, I'm not sure that they invariably wait that long to do serial mag threes. And then you really, once again, have many, many surgical options and these depend on the size of your patient. Are you dealing with a newborn? Are you dealing with an older child? The size of your ureter whether or not there is currently infection? Um and really also what you're comfortable with. So for your Children who present, you know, with an acute problem. So, um for example, pyonephritis, um I think a nephrostomy tube um is not a bad option, you know, um they're actually perhaps not that difficult what to do if you've got interventional radiology at your center and you're not kind of comfortable with ureteric surgery. Then um the nephrostomy tube is um obviously always a safe option for Children who need urgent decompression, even those who are, you know, too sick to go to theater. Um A cutaneous urostomy is something that I favor quite a lot in um young babies. Um stenting. Um not something I've ever done, but there are centers that do it. I'm not sure. It's, yeah, it's not an option for everyone. Uh We'll talk briefly about the refluxing reimplant. Um And so these are really for decompression. So these are tied over a patient who has either had an infection or is perhaps too small for um a definitive reimplant. So your definitive surgery would be your ureteric remodeling and a reimplant. And then Toronto use the side to side um urethro um cystotomy as um definitive surgery once again controversial. So, stenting. Um so this is, you know exactly what it sounds like. Um cystoscopy and placement of a double J stent through the ureteric orifice. Um It has been like I said, done in some centers. Um II think it's really not as easy. Um So you have to really have um very small stents available and often that distal segment of the ureter and the uo like anybody who knows who's done a cystoscopy for some of these patients will know that that ur is often really, really tiny. Um And it's really difficult to get a stent in. Um the problem with these is that um you know, the stent needs to stay in for like a good um kind of 6 to 12 months. Um some of them will need um recurrent stenting in that time. So it involves like several anesthetics and a fair amount of ionizing radiation um because you're supposed to do it under fluoroscopy. Um although half of these patients ultimately no longer need surgery, um about a third actually have complications. So the stents migrate and fall out. Um like I said, you've got repeated doses of ionizing radiation and anesthetics. Um Some of these patients do actually ultimately end up um with lots of renal function. So, um, deteriorating renal function on the, um, the mag threes and some of them actually ended up with nephrectomies. And then, um, the other thing is that, you know, in those patients, so you're kind of 30 to 40% who do require further surgery. Um, you know, the stent really does inflame your ureter and make that surgery, um, more challenging. Um, this is probably not really an option that I would opt for in small babies. Um I think perhaps like uh an older child where we're just trying to temporize them. It's, you know, not a bad way to go, but probably not my preferred option for a neonate. So, cutaneous urostomy, um like I said, uh I like this for small babies. Um I have to say for especially for pediatric. So I think, you know, pediatric surgeons are often intimidated by the ureter. But for those of us who are kind of, um, you know, very used to doing, for example, um descending colostomies for patients with anorectal malformations. This operation is actually very, very similar. Um The main difference being that you're doing it through a Gibson incision and you're trying to stay extraperitoneally. Um But then yeah, you can bring a little loop um cutaneous urostomy. Um It's not a huge surgery, the babies recover very quickly after it. Um, and advise you lots of time. So if you've got a nice functional urostomy and a decompress system you can need your baby until they're about a year, year old and then go and do your reimplant. Um, stomal stenosis is a well described problem. Um And some of them need revision. Uh Stomal pro prolapse perhaps. Um, not so much a problem. The ureter might still not drain after this procedure as you saw in that ultrasound that I showed you. The ureter can be really, really torturous and um sometimes just opening it here is not enough because the ureter in addition to being very dilated is aperistaltic. And even though you've opened it, um it might just kind of collapse on itself and not be able to empty it. This is also really not a particularly good option in patients with bilateral disease because you end up defunctioning the bladder, which is not ideal. Um So bringing up donors bilaterally, um you leave a fallow bladder which then doesn't cycle um and can be problematic later on when you kind of want a slightly bigger bladder and also a functional bladder to do your reimplant in. So this procedure, I personally don't have experience with um the tr ur um which was described in Indiana, which is temporary refluxing urethral reimplant. And essentially, um this is once again for babies who are um either too small or too sick for a full um tapered or remodeled reimplant. The idea being here that you spare the patient a stoma and then in bilateral disease, you're still going to allow the, the bladder to, um, uh to cycle and to, um, to fill. Um, essentially what you're doing here is saying that you're prepared to trade obstruction for reflux. So, um, you know, I think most of us would agree that obstruction is worse than reflux. Um, it's higher risk, a higher risk for infections and loss of renal units. Um, with time, I think many of us are becoming less and less concerned with reflux. Uh But what these guys do is essentially take the ureter and kind of plug it onto the dome or the anterior aspect of the bladder. Um This will be a refluxing reimplant, there's no tunnel. Um and it yeah, uh kind of t your baby over until once again, you can do a, a formal reimplant. This is not intended to be definitive surgery. So, um in India, when they do this procedure, they go back a year later and reimplant the patients. Um I just wanna say like there is a nice video um uh like Martin for describing this procedure. Um and he is the pain to say that it's a very easy operation, but he really do does recommend that you watch the video, um which is available um first before attempting the surgery. Um just because there are a couple of, of key points that you need to know about specifically, I think about kind of preserving the blood supply of the ureter. And um the resection of a bit of redundant, which is sometimes done. Um So they feel that with a procedure, they have a really low complication rate. They feel that um you know, the infection rate is about 35% which is probably equivalent to all Children who have primary um obstruction megaureter. So about a third of them will get an infection still. Um But they have um really good outcomes with this and they're very happy with this operation. And it's also um a good option for Children who have a single functional system. So, um an absent contralateral system. Um So to talk about the uh reimplant, which is once again the most conventional and definitive um procedure. Um The objective of your surgery is to um reduce the ureter caliber um to reduce your reflux. But to allow emptying, you wanna protect the ureteric blood supply and avoid damage to the bladder. Um Just a couple of technical notes, um you know, the tunnel length should be long enough um to uh uh accommodate the diameter of the ureter. Um The hiatus of the, the ureter shouldn't be lateral and you should try to avoid things some angulation. Um Just, you know, things to think about when you're dealing with a, a megaureter is that you can't just plug this giant ureter straight back into the bladder and you need to taper it a bit, you need to excise this distal narrow segment. And then depending on the size of the ureter, you can try to just um fold it or taper it like this, um which theoretically helps to preserve a bit of blood supply. Um But there's a magic number of 1.755 centimeters. Um If it's wider than that, you actually need to consider resecting a bit of ureter, um which can lead to blood supply issues. Um But yeah, your tunnel shouldn't really be longer than you tapered or sected um section. And then once again, you kind of really want to do this in a bigger baby to reduce your risk of bladder damage and um other complications. And then I'll just touch on the side to side, um Urea cystostomy. So we did a lot of these in Toronto and I have to say once again, it's a very, very easy operation. Um And once again, it, it, it's also just a refluxing um procedure um where you once again, um uh ovulating the obstruction, but you're allowing some reflux. And really, they do a small, tiny, like you can see here, this incision is maybe two or three centimeters. Um in the right lower quadrant, it's usually ultrasound guided. So they fill up the bladder and then have a look at where they see the ureter and the bladder touching each other, do a little incision and then really just do a little side to side anastomosis leaving the native UO as it is. Um So like I said very simple operation. Um You can do it in very small babies. I think the average age of patients in the study was three months. Um babies go home very quickly. Um They sometimes get UTI S once again as always. Um But some of them, yeah, it, this is intended as a definitive procedure. So the Toronto group has no intention of going back to, to a a reimplant in these kids. It allows reflux and the dilation goes away. Um But some of these babies do go on to um require definitive replant anyway, because they have um recurrent infections from their reflux. And some of them actually also get stenosis of the anastomosis and get recurrent obstruction. Um So yeah, my final words in urology, there's never a single right answer. And I think um it's always best to do what is safe and to do what you know, um and to ask for help or advice when it is possible. Um I think, you know, we always present all of these things as very easy options, but um all of us who do these surgeries know that these patients do come back with complications. Um Yeah, and I'm happy to take any questions current. Uh Thank you very much. Uh It's quite a vast topic and, and pediatric urology is all uh interconnected, upper tract, lower tract bladder, everything is interconnected. So you gave us a very nice wide overview of ureter abnormalities and I, I think your last slide is probably the most important that uh you do what uh you think is safe. And also you highlighted that many of these dilated systems, they just recover with time. And um and that's, that's quite an important message. Uh And, and don't look at the dilatation and uh think of operating and also try to avoid it intra cycle operations in young babies up to first year uh uh of in the first year of life. So I think these are quite important messages. Um If, if uh I just ask doctor Yoda Mann, our consultant, pediatric surgeon to give comments because in five minutes time, she's likely to have load shedding at her place and may not be able to comment. So. Yes. All right. Thanks pro yes, definitely load shedding at 6 p.m. So II thanks Karen for the for the talk. It was really good to get the overview and um the different surgical options. Um I just uh we don't see a lot of primary megaureter, but what we do see is the Eagle Barrett or the prune belly syndrome patients. I wanted to ask if there's any specific advice you would give for, for following those patients up and when you, it doesn't differ uh uh the indications for surgery for them because they progressively get dilated and, and obstructed. Yeah. Yeah. So there are a particularly difficult group of patients also because there may be um kind of in that fourth group of like nonrefluxing, nonobstructing and that their dilatation is often kind of related to their global kind of lack of abdominal musculature. Um I have to say, um we tended towards the fairly conservative with them in, in Toronto. So kind of trying to ignore ureteric dilatation so much and rather focus on kind of upstream issues. So whether or not they were getting infections and then progressive hydro and parenchymal thinning. Um a couple of patients, you know, they don't have valves but they've got, you know, kind of a lower um a urethral um sort of um hyperplasia almost and a couple of these patients do end up with um vesicostomy, um which doesn't always resolve the hydroureter. Um We were pretty reluctant to reimplant these guys um, too soon. Um And often sort of waited until they were really old. They, you know, they really had to earn their um, yeah, the ureteric surgery because, you know, once again they're kind of a type where, you know, the ureter is not always the primary problem. Um Yeah, I don't know if that answers your question if they're a really difficult group of patients. Um And I think, yeah, you kind of take them on a case by case basis. Um But yeah, you know, a subgroup of them do progress to renal failure and um you do kind of wanna prevent that where you can. Ok. Thank you. We might call you about specific patients. And uh uh I, I'm sure Karen uh would not mind we asking her a advice for a specific patient. And Karen, what I also like was uh to see that you advocated uh ureteroscopy. Um I thought that that operation had gone into disrepute. But uh because that's something which uh we had done in Mumbai long time ago when I was training, my consultants had done that operation and it really tied over the crisis and uh Children uh lives could be saved. And yeah, I think that is something uh important for young pediatric surgeons to know that uh we are used to doing colostomy. So it is uh one can do a ureterostomy. Uh and then you can decide to refer the patient to uh to a more uh experienced and competent surgeon. So I like that you mentioned about urethrostomy as well. Yeah, it's sort of interesting for me because I think most of us, you know, consenting a parent for a colostomy is like second nature. We all know it's going to be ok. Um It's a temporary thing, but there's something that feels worse about telling a patient you're gonna do a ureterostomy. And I think it's because we don't do them that often. Um But, you know, for me, it's also better than a device. So if I have a patient where I have a choice between um you know, doing a stoma or um a situation where if I put in a nephrostomy tube, I know it's gonna be there for a long time. I would, I would really prefer to go for the stoma because the nephrostomy tubes give a lot of problems. They block, they fall out, it's difficult for the parents to manage them. Um, they get infected. Um Yeah. Yeah. And I have to say we did, even in Toronto we did a fair number of ureterostomy um for like a, a really a very um kind of patient group. So for lots of different um indications, but it was definitely something that we still did. Yeah, I think it, it is very important that uh you brought this point out and that is an operation which uh uh a competent pediatric surgeon will be able to do and uh will certainly be able to tide over the crisis. I see Pro Seal joined a little bit later, but I'll ask him if he has any comments. Uh Professor Seul, is uh Professor Saro still here or has he left? Um is here? No lenny. Can you unmute yourself, please? I'm not sure whether he's able to hear me. Uh I saw doctor Malou was here, ILIT um or struggling to unmute. Uh Yes, ma'am. II am. Yes. Neli can you unmute yourself in the meantime? ILIT? Do you have any comment? Uh No, thank you very much. Prof it was a good presentation of Lord. Thank you. Ok. Uh Pro syphilis, enjoyed the presentation. Are you able to unmute yourself? Now, it looks like uh he's got uh is this problem to unmute Karol? Uh I will ask you uh to give like a final comment, take home message. Um And I thank you for your presentation. I was gonna ask, I saw Prof Lazarus here earlier. I think maybe he's gone. Now, I was gonna ask him if he had any um comments. Um But yeah, I think we're always, you know, we're a small community here. So we also are always um happy to um answer any questions or on the phone. Um Yeah. Um yeah, we always happy to be available and to um give any kind of advice that we can. So thank you Karen. I think uh that that's good to know and uh I think this is the beautiful Nelson Mandela Children's Hospital. So those of us who haven't visited it, it's now fully functional. So when you are in Johannesburg, next time, either talk to doctor Karen Milford or doctor Andrew re and they will be happy to show you around. And uh yeah, we, we love to have visitors and, and yeah, we really do. Um I'm always happy to have anybody who wants to come and say hi, yeah, we visited when it wasn't fully functioning. Uh I think only one word was functioning but it is nice to know that it is fully functional now. So, no, thank you once again. And Thank you for having me. Next presentation will be on the Tuesday seventh of December, uh doctor Ash ma consultant, pediatric surgeon in Durban. He will give a talk about HIV and pediatric surgery. So that's going to be quite an interesting talk and that will be the last talk for the year. So we will uh post the invitation for that meeting uh a week in advance. So thank you everybody. Um I wish you well and we see you in a month's time. Bye-bye. Thanks. Bye.