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The recording has stopped. Hi, how are you? Can, I'm going to see my screen check that everything's working. Uh Yes, great. Um You can share your screen and then we can just check your slides. I can see the bubble reconstruction slide if you just want to go back and forward on slideshow. Is that fine? Um No, I'm still seeing the Yeah. Ok. That's fine. Yeah. OK. I can see the first light. Um Yeah, you can just go forward and backward. Um Yeah. No, if you can um project the slide show uh uh view because here I'm able to see the next slide as well. Oh, I see. But I've got things around. Um Let me do that. Hm I'm still seeing your next slide. So you just need to change the the um are you using windows or uh apple windows windows? So, um um are you in powerpoint? Yes. Or you can just go to um slideshow and you can save start from the first slide from the beginning. It's still showing that view showing that. Um Let me just go to display settings. I think it's probably there we go. There we go. That should be fine. Yeah, that's perfect. Yeah, that's perfect. Let's see if you can go forward and backward. Yeah. And you don't have any videos, huh? Ok. Ok. That's, that's easy. OK. Now you can uh maybe just go to your first slide and, and stop sharing. I've got those uh uh there's one slide of of introduction. OK, then I'll stop sharing again. Uh We start in just about five minutes. Excellent. Ok. Thank you, Marian. Can you see my first light? Yes, I can. Ok, start. Now. This meeting is being recorded. Good afternoon, good evening, good morning. Wherever you are joining from, welcome to the Zoom Academic Meeting of the Department of pediatric Surgery uh in East London, South Africa. And we are very happy uh to welcome uh Doctor Marion Arnold, uh who is going to give us an interesting talk about clinical dystrophy and African perspective. Doctor Ard is a consultant pediatric surgeon at Red Cross War Memorial Children's Hospital affiliated to the University of Cape Town in South Africa. She I actually literally followed her career and it is so nice to follow her from when she was a medical officer. After completing the basic MB CB degree from Pretoria in 2004. She subsequently did her fellowship in pediatric surgery and me from Stellenbosch University in 2012. She also did a colorectal fellowship for six months in Cincinnati um where she worked in colorectal and pelvic reconstruction uh surgery and also vascular anomalies and naturally. Her special interests on neonatal surgery, pediatric colorectal surgery, vascular malformations, and palliative pediatric surgical care. She says that she has no time for hobbies as she is a busy mother of two small Children and her favorite line is without courage. We cannot practice any other virtue with consistency from Maya Angelou. Um Marion has uh been assisting us in state and private sector with all complex colorectal and vascular malformation cases. So Marion, welcome. I will stop sharing. You can start sharing and give us your talk. Thank you very much. I think uh my first reaction when you asked me to uh talk on this topic was that I'm hopelessly um not, not, not the right person, but the reality is, I think unfortunately, most people do not have much experience with, it is extremely rare. Um So I'm going to be uh talking about an elephant of a topic um both in how it appears but also in, in terms of the challenges it presents to the pediatric surgeon to the patient. Um And I'm going to try and talk about it from a low and middle income country perspective. Um But I think the reality is that even in high-income countries, there are major challenges with the management of this um condition. I'm going to talk a bit on um medical and surgical aspects of the management, but it's a huge topic, there's very little time. Um So I'm just going to give sort of an overall approach and just mention some of the challenges of long term and multidisciplinary care. It's by no means a uh sort of overarching comprehensively um covering of the, of the, of the topic by any means. Um But I think it's, it's good to talk about these topics because that's the way we are going to move forward in finding better ways to manage these patients. So, exstrophy comes from the word turn inside out from the Greek. Um And you can see the typical elephant, um trunk appearance with the elephant ears of the bladder hemi plates. And that is um the appearance of this condition. Uh It's often not diagnosed antenatally. We'll talk about that a little bit more detail. Uh And it's characterized by uh the appearance of the um visible mucosa of um two bladder hemi plates uh with the sort of um feet cecal and um sort of plates with the prolapsing terminal island are often present, not always prolapsing, but um often is. And then there's another opening, often from leading to a very shortened for shortened hind guts. Um just below that elephant's trunk. Um Sometimes the gut can even be completely um absent and it's, it's so atretic. Um but usually there are at least uh sort of, you know, five centimeters or so that you can find, um there's an imperfect anus, there are spinal defects in almost all cases and it's also associated with an epispadic um um phallus um often to hemi falls, in fact. Um and in case of um female patients who are slightly in the um predominance, they, you can have uh extra feet opening of the vaginas. Um two hemivagina usually um also just beneath these. Um the, the, the bowel and the bladder place, it's part of the bowel, the bladder exstrophy coal complex. So the um the sort of troph epis complex as it's known. Um but bladder ECs, although there are many um aspects which are um in common, um is, is definitely a lot easier to manage uh by comparison to because it doesn't involve quite so many systems. It was previously known as vasointestinal fissure if you're looking for old literature on the topic and other things that are um also seen in this condition, uh a low umbilicus and a single umbilical artery together with a wide eyelid crest and very widely diastase pubic aus. Um These things are relevant when you might present with a patient. Uh It's got a variant known as covered cloacal ECs uh where you've basically got a patulous bladder neck and, and various sort of um aspects of the, of the condition, but not necessarily the abdominal wall defects to the same degree. Sometimes you just have skin lying over over the bladder. Uh They also usually associated with at, in males, um and inguinal hernias very commonly and many cases also for short, um small intestine, although sometimes it's uh more of a functional thing than an anatomical short intestine. Here, you can see a schematic of the condition, but if you think of an elephant with its ears and its trunk, um you, you will be able to appreciate um, the sort of, and, and remember that that schema here are a few examples. Uh The bladder plates are sometimes completely separated by the bowel plates. Sometimes they're confluent above it, sometimes they're confluent below it. Uh And um you can see varying degrees of prolapse. And as part of our pharyngeal diagnosis, you can see a picture of uh the normal bladder extrophy, which is far more common and it, it's most likely to be what you are being referred. Uh If you've got a patient presenting with a visible mucosa on the abdomen, um uh Racal troy with a prolapsing bladder, um sorry, uh Racal sinus or, or prolapsing bladder sing through a patent rac can also present with a, a similar picture. So those are your main differential diagnoses. Parents always want to know why did this happen. Um There are no clear um reasons we have got a few suspects, uh candidates genetically wise, but it seems to be quite multifactorial smoking has been implicated, but essentially around about the fifth to eighth week of gestation that has been the development of these important structures takes place. And when you have abnormal um ingrowth of the mesoderm, uh that makes up the, the, the the sort of um mesoderm that is supposed to grow in between the ectoderm and the endoderm and the coal membrane and the fifth week of gestation due to some intrinsic abnor abnormality of that mesoderm. We think um if that takes place before the urogenital sinus has um properly developed um by the descent of the dental septum that can lead to premature rupture of the cloacal membrane. Uh which is thought then depending on the timing of that rupture to lead them to uh cloacal ECs in the most severe form or bladder ec or just epidius. It's pretty rare. Um depending on whether you include live, live birth or, or stil as well. But um estimates anywhere between um sort of around about um one in 200,000, maybe one in 150,000. Um more common in females and antenatal diagnosis probably on average about 25% in first world countries. It seems by going up to 90% in some series. Unfortunately, as you all know, in low and middle income countries, that incidence is much lower. Um Sorry, just the things that can give you a suggestion of not being able to visualize the the full filling bladder and you can actually see the prolapse highly and visible on the um on the scan. But things like uh widely um people also will give you a clue. So survival was historically dismal. Um And uh series at um Red Cross here where I work in 1986 8% survival. Um in A NM IC series from India in 2013. Um this over half of patients surviving. But in first of all countries, us um reported over 90% survival um as early as 1987 what kills these patients? So, nutritional things um related to the high output stoma. Um continence issues are obviously not so much a lethal thing, but uh they can cause massive social stigmatization, stigmatization um which might lead to abandonment by parents, which is um often um leads to other other problems. Renal preservation is a major issue. So, recurrent urinary tract infections um as well as surgical procedures that we um perform, which can then lead to uh obstruction of the um of, of the bladder um and hydro nephrosis and um ultimately endstage renal disease um together with infections. Um We'll talk a little bit about major ethical issues associated with historical practice of um preferential female um gender assignments. Um But there are also significant sexual and reproductive challenges that these patients face which can contribute to poor quality of life. Um quality of life um is significantly impaired in these patients long term. Um There's also increased risk for things like cancer which is also associated um with, with what we do surgically to these patients. So, um major challenges uh for these patients. Um Here you can see a photograph of um from a report of a patient who, um, had sort of partially um, surgically repaired, uh, fatal sy, there is a long term results of an unrepaired bladder plate. She's got a diverting colostomy. Um, she had a part of um, the bowel used to create a neovagina. Um, but uh patient has got severe neurodevelopmental problems. She's due to spinal associated spinal anomalies, she's not able to walk, um, and very poor outcomes. And, you know, sort of a lot of ethical aspects are po when you look at the long term outcome of these patients, which we need to be mindful of when we're making decisions in the neonatal period. Um Here, you can see a patient presented in the neonatal period. Um a bit of a late presentation, you can see significantly prolapsed bowel, um plates there. Uh And I think most markedly is that the child was an extremely poor nutritional condition. Um And this patient, uh according to the reports did not make it, but I think you need to consider that the nutritional status of this child um is, is really, really critical to determining the outcome. I think we sometimes forget that this is not the same as gastroschisis where you've got exposed viscera. Uh Although long term exposure of the viscera can impair the outcomes, uh there's no rush to close the defect as there is in gastroschisis. Um So the principles are a stage surgical approach in the most part uh one stage surgical approach has been described uh where everything is um closed at once um with um sort of good outcomes according to, to some reports. But um there are significant disadvantages of, of that approach. And I think in our setting, um where we often don't have the ICU support for an aggressive abdominal closure early on, um that's going to put your patient at very high risk of abdominal compartment syndrome. Um And um if you've got any leak from an initial um suture, closure of that um bow place, uh you're also going to be at high risk of of sepsis and associated um death. Um So a far more conservative approach, I would argue um is is required in a particularly in a low and middle income setting. Um So typically, classically, there are three stages to repair many patients only uh go through stage one and two. So the first one would, would be closing the abdominal wall defect, um including closing of the bladder plates and creation of a stoma um followed by uh procedures to address continence and genital reconstruction at a later stage and in a small number of patients, um it has been shown that it is possible to improve um fecal continence um through um bowel management measures and consider um doing a anorectoplasty for for patients uh where successful bowel management has been demonstrated while they still have a stoma. But before we jump into operation mode let us first have a look at our patients. So, comorbidities, we don't see um quite the same spectrum of VL spec spectrum associations that we are used to seeing uh with patients with anorectal malformations. But many of them do have potentially fatal cardiac abnormalities that do need um a cardiac review echogram um on um initial presentation, uh problems, respiratory rise with impaired respiratory reserves due to um pulmonary hyperplasia may be present. Um Almost all of them with modern MRI um are shown to have spinal abnormalities and many of them um probably about 75% of them severe spinal abnormalities such as myelin and so on. So those require consideration um and may require urgent uh more urgent um surgery to treat uh to address the spinal anomaly than to address the bowel and bladder concerns. Um You should also get a baseline renal ultrasound. Uh Many of them, most of them have, have got um normal uh preserved upper tract initially, but you need to just um confirm that so that you have a baseline to compare to after you've done a a procedure to close the bladder, which can often cause hydronephrosis down the line. Most patients in a low and middle income country setting die of sepsis. Um So it's really important that we look at how we're managing that omphalocele. Um So typically that would be managed just with um something to keep it um from getting sep sex like a topical agent. We use, uh acticoat flex, which is a silver based, um, dressing. It's quite expensive. Unfortunately, um, many people often just using, um, sil silver sulfADIAZINE, um, as an ointments to apply onto the omphalocele. Uh, and prophylactic antibiotics also need to be given. Prophylactic antibiotics should be given, um, until the patient has ureter reimplantation because that you've got this exposed bladder plate, but also, um, refluxing ureters inserting into the bladder plate. Um So the patient is very high risk for sepsis, both from a urinary tract infection point of view, as well as from sepsis of, of that omphalocele. So the group of or recommend waiting until the patient is at least 2.5 kg and gaining weight before any surgical procedure. And I think this is really good advice. Uh How are we going to achieve that though when we've got a patient who's essentially got the equivalent of an ileostomy, often a high output ileostomy. Uh So we want you to promote bonding. Um So let's stay away from IV fluids and medicalization, breast milk. Uh that might need to be fortified. Um loperamide to help slow things down and improve absorption. Sodium supplementation is absolutely critical in patients who've got uh in most neonates who've got um uh ileostomies. And uh we monitor the serum sodium. Uh sorry, no, the, the urinary sodium of our patients on a weekly basis. Um until that is um optimized to ideally more than 20 to 50 Miol per liter and to make sure that we, we do our best to get these patients anabolic, um, in a lot of overseas literature. Um, patients who've got, um, early, um, operations, um, can take up to sort of five weeks sometimes to get off parenteral nutritional support. Uh, but I think there, um, is, you know, there's no reason to rush with the surgery, um, and rather to wait until the child is in a good nutritional state, much lower risk of wind breakdown and so on. And this can usually be achieved with enteral um means and, and not parenteral nutritional support necessarily. But attention to detail, particularly with the sodium supplementation um is is important. Then if you're not rushing to operate and you first want to make sure that you've got uh surgical expertise, appropriate surgical expertise, perhaps referring to another unit, um et cetera as well as uh optimal pa patient condition for surgery. In the meantime, you've got to protect the surrounding skin. Um And you've also got to protect the bladder plates. Um So diligent irrigation of that bladder plate um protecting the surrounding skin um with um some, some uh protection um of whatever you've got available. Really. Um So uh you can um use your or your usual barrier means that you use for um around. Um but the bladder plate is recommended to use something like cellophane or soft plastic, basically just to reduce irritation from the, uh, from the Nappy which can abrade and, and cause, um, otherwise increased fibrosis and, and ation tissue on the exposed plates. And during this time, I think it's important to engage with, uh, parents and understand their expectations, um, and manage their expectations. So when, uh, you are, at the point where you think, um, you can now do surgery. The, the classical, um, first stage of surgery is actually quite a big operation. And I think it's important to consider that depending on what you are dealing with, um, it might not be the best setting for that patient to jump in and do all of this. It has been described to just do an initial ileostomy, um, only, um, separate the, the bowel, um, away the fecal stream away from the, the bladder plate, um, and then come back to do the rest of the abdominal closure. And I think that's a very valid approach. Uh, but the, the traditional de um, description is to attempt to close both the omphalocele, the, the bladder, um, plates and the bowel all at once. Uh, so that is approached through initial ureteric catheterization, um, just to help protect the, the ureters and make sure that they are not injured, not always so easy to find them, especially if it's a slight age closure and the bladder mucosa is, is quite, um, sort of a bit bumpy. Um, and if you've got a very delayed closure and you've got, uh, bladder plates which are very small. Uh, then it's advised not to necessarily attempt to close that. Um, but rather wait until the child has grown a little bit, you could consider first to do an ileostomy. Um, and just let everything else be, um, and wait until the child's bigger. Initially, it was thought that the bladder plates are not going to grow. Um, but if you, if you wait a few months, um, it is possible to do a complete, to, to do those sort of um, either a single stage approach to close everything, um, or um, potentially. Um So, so you can basically have a conservative approach to managing the omphalocele just allowing um a um, and then address the bowel later or you can do deal with the bowel just divert it initially and, and then come back to, to, to manage the, the bladder place a few, few weeks, two months to several months later. But as I said, the classical approach is to do all of these um things at once. Um So the bowel is separated um, from the omphalocele and then you can tubularise over a catheter, you can tubize the iron. Um Oh, sorry, you can get the reduce the iron tubize the cecal plate and then bring out a colostomy, which is a lot easier than it sounds because the uh the p gut is got an abnormal blood supply. It's very, very fixed and tender to the posterior abdominal wall. Um and that makes bringing out a colostomy in these patients actually very challenging. It's usually um you barely manage to get the length to the skin. So there's a very high rate of colostomy complications described in these patients. And if you look at um many of the, the, the largest series, probably about 50% if not more of these patients um initially are managed with an ileostomy. Um Sometimes that's just um a temporary situation to allow your suture line if um to, to heal um over where your tubularized um cecal plate is and then that's closed a few weeks later. Uh But the reality is the incidence of hi got loss from vascular um inadvertent vascular misadventure, I think is, is quite high. Um Although sometimes it is because the patient actually doesn't have much fine gut, but there is often about five centimeters or or so of hi gut there, sometimes a little bit longer. Sometimes a normal bowel length has been described but not so often. Um But the idea is that if you want the optimal long term nutritional outcome, you would like to preserve that hind gut. Uh so that you can later uh connect it to the fetal um stream, potentially, it can be done in one stage. Uh But the the the chance um of breakdown of that um cecal plate tubularization needs to be considered. So you might need to consider bringing out a covering ileostomy and um then decide to close that later on. The things that you need to consider um to facilitate the success of this are also whether you need to be doing concurrent osteotomy. So if you are doing an early neonatal repair within the first three days, uh even sometimes up to up to a week or a month, it has been just to use a nylon stitch to approximate the um pubic grandma and improve, reduce the tension across your um across your your bladder plate and allow better reduction of your bladder plate um and better less tension across the abdominal wall. Um Over 72 hours, it's recommended to you consider the use of osteotomies um to improve your um the the to reduce the the risk of breakdown of particularly your bladder repair, but also your abdominal wall repair. Um So I think that is, is something once again where you need to have a multidisciplinary team discussion and look at what you've got available in terms of expertise at your institution. Uh Many patients do not achieve uh primary closure of the abdominal wall. So here, for example, the child with a massive omphalocele had a stage reduction using mesh. Um Many um particularly um low-income settings do not have mesh available um and consider to preserve the omphalocele membrane at the time of surgery. Um I would in that case recommend just lifting it up and then taking it down again. So don't try to incise it and expect it to heal like normal tissue that's not going to work. Um But obviously, you need to consider how that is going to be managed in the context of sepsis and proximity to the fecal stream. Um So try to get your diverting stoma um far enough away that you can, you can manage that. So the principles of approach from a urological point of view, I'm just going to give a board overview. I don't do much urology myself. I always work with. Um I'm fortunate to have a person who is a dedicated pediatric urologist at my unit. Um So we always work together on these. Um but um most patients don't have significant major underlying renal problems, although they do need to be screened for that. Um pelvic um and your kidneys and so on have been described. However, and sometimes they do have um uh renal ipsilateral or one sided um renal agenesis, uh initial bladder closure, as I said, was traditionally done in the neonatal period, but can be deferred to be done later on, often done about 6 to 12 weeks of age or with within that, that first period. Uh but there's a high rate of failure, um even under ideal circumstances, up to 28% of them described to, to fail. And many of these patients need multiple re attempts. And if they, if it fails, just pause. So you should optimize a patient and wait at least 4 to 6 months, preferably between attempts and definitely consider doing osteotomies um which can be staged, done a few weeks prior, possibly with improved outcomes. Um depending on your, on your situation. Um It's important to a after initial um bladder closure to, to screen for subsequent hydronephrosis which can occur in um 1/6 to a third of patients. Um And then typically, um after initial bladder closure, the next step would be um particularly in male patients to consider um look at, looking at penile reconstruction, um sort of at the end of the first year and then to start um worrying about bladder neck ruction, um or or reconstruct your bladder neck procedures for um your patients to get the incontinent only around about the age for more or less of five years. This will depend though on the bladder plates. So, a patient who's got very poor bladder plates, not amenable, bladder, neck, bladder, um plate closure um to allow um cycling of the urine um with within that um that bladder um you might need to consider to do a um external um external diversion, incontinent diversion, typically with an ideal conduit. Um And then uh for older patients who've had a successful uh bladder closure, um they can be considered for bladder neck reconstruction. Um sometimes at the same time as the as the penile reconstruction. Uh but majority of patients with bladder with um local troy as opposed to bladder extrophy will actually end up with an augmentation um typically done around about five years of age to get them clean. And there's only really one report that I could find of a patient who was actually spontaneously voiding um without C IC. So a majority of these patients are going to need clean in catheterization, usually via an um a Monti a continent Conduits or an incontinence um Conduits. Um in the majority of cases, ureteric reimplantation, does it needs to be done at the time of um either bla bladder neck reconstruction or um at the time of um augmentation because they all have um they all have reflux and then um the ial hernias and other testes also needs to be addressed. Unfortunately, long term, the the underlying bladder in these patients is abnormal. So they tend to have uh progressively um more dysfunctional bladder over time. Neurogenic bladder is um definitely worsens. The outcome both from a urinary tract in uh point of view, as well as from a bowel point of you have got increased um increased risk of recurrent sis um uh with, with neurogenic bowel. Um and long term, they can have urinary calculi, um particularly with small bowel augmentation as well as obstruction um related to the uh the the surgery. So they definitely need close observation. Chronic kidney disease is, is um common, common um and needs to be monitored for um with one patient being um developing renal failure, needing renal replacement at the age of five years. And then long term, they need surveillance for bladder malignancy, which is up to 300 times um more common in these patients. And with the incidence in adults of up to 17.5%. So they need long term surveillance for that. Um I've just mentioned a little bit about um possibilities for for um urinary reconstruction. Um And unfortunately, most of the, the textbooks are talking about bladder exstrophy patients, which is completely different, different kettle of fish compared to critical ex. So, um these patients do not have the option of, of using bowel usually um for their um their bladder reconstruction. Uh although the the thinking on that is also changing. So there, there was a report recently of a patient where at the time of initial reconstruction, the the um extra feet, um bowel plate was left on the bladder as an autologous um augmentation of the bladder. And the patient just had the, the hind gut um and the, the ilium um separated, leaving the, the cecal plate in place. Um So something to think about. Um but um they, they have historically been concerns that that will lead to absorb reabsorption of um the urine. And um these patients might get metabolic acidosis. Although that particular patient, it wasn't borne out to to be. So probably it's more the sodium that patients need than necessarily always the bicarbonate supplementation if they don't have significant short, short bowel genital reconstruction. Um It's been fraught with controversies um to the extent that patients have historically often been signs uh female gender. Uh but improvements have been made and um surgical reconstructive technique still very high risk of corporal loss um for patients particularly in, in the hands of somebody who's not um doing this often. So I think this is something that should really be done in 10 of um high volume um and excellence. Uh these patients typically need a lot of revision surgery. Um I was describing about percent of patients since it's in high volume series, they still have um all of them have sexual dysfunction, um very poor outcomes cosmetically with uh which is congenitally. Um only 50% of normal size and um various things have been tried. Um Some patients can get forearm flap sort of rescue procedure later on as um older, older patients sort of in adulthood. But even in the bladder dystrophy population, in some reports, only 50% of them are actually ss active due to concerns about the the appearance of their genitals. Um So I think this is something that, that needs to be um done by expert hands. So it's not to make a bad situation worse. Um and also managing expectations of the parents as to what is realistically achievable in these patients. Uh I is basically what's going to be required from a fertility point of view for these patients, uh as I mentioned, historically, um these uh patients who were born um 46 Y with to extrophy were often um automatically gender reassigned as, as females often without even letting the parents know. But that's been shown not to be uh a good idea from a um point of view of neonatal imprinting of the or antenatal imprinting of testosterone. Uh So, in a long term outcome, uh 11 out of 51 patients who were, who had male gender or female gender reassignment were actually living as males with a further seven reporting gender dys. And in another study, 55% of them actually sought sex reversal um surgery in a in another study. Um And we also have better penile reconstruction techniques now than were available historically gynecological for 46 xx individuals. Uh Most patients have got um two hemi vaginas typically with the um didelphys uterus as well. Some of my patients in up to 25% though can have an element of agenesis. Um And so various uh vaginal reconstruction techniques might need to be considered this. It's usually done at the same time as the urological surgery in the neonatal period, but it might be need to be done later on. Um Depending on what the underlying problem is. Uh Once again, I think care needs to be um made to, to not necessarily use uh so-called useless hind guts um which might be a amenable to be um restored into the fecal stream. Um I remember seeing a patient uh who had uh was actually in the 46 xy um individual had been um received neonatal female um gender assignment and patient here was very distressed by a massive um very smelly. Um this uh big uterus, sorry, big um vaginal uh or near vagina, it had been made using the um the high cut remnants um which is basically complete precluded any further reconstruction for that patient. Um And um I think we need to be careful of the decisions that we make um at a young age for patients um with which could perhaps potentially in some cases rather be deferred. Um So just a thought, many of these patients really struggle. Um despite reconstruction with um issues with genital prolapse, as well as dyspareunia due to the abnormal and uh position and very short uh vaginas. Um But normal fertility has been reported although with a high risk of premature labor um and planning for Cesarean section needs to be made. I've mentioned many of the issues related to bowel reconstruction. So I think um although the textbooks all say you should doing everything all up at once, I think one does need to carefully think about whether they're doing an end iost toy. Um possibly with a he got separation. Um Well, certainly with um separation as, as being the traditional way of doing things. Um but possibly um consider other alternatives depending on your surgical expertise at hand. I think the, the important thing is that you do want to try and um that bit of, of colon that that is available. Um You do want to try and join that up into the um fecal stream. It has been reported that um saline enemas have been used to dilate a separated off gas and then later uh added onto the ileostomy. Um So the things just to bear in mind if you do have a tubularized cecal plate and a colostomy in the context of short bowel, that might um be associated with poor motility of that cecal um area bacterial overgrowth is common and you kind of increase predisposition to oxal the bladder stones. So, um oral in bicarbonate as well as calcium supplementation needs to be considered. Um And the reality is that only about 50% of patients are amenable to future anorectoplasty in an ideal circumstance. And that's where you've got um high availability of so um soluble fiber and so on to, to improve bowel um consistency. Um So, uh you need ideally, you know, older patients more than 20 centimeters of colon for that to be possible for, for uh anorectoplasty. Um But certainly adding even a three centimeters of colon um to an uh ileostomy to create an end colostomy can improve nutritional outcome, nutritional outcome. Still though, um not always that great in many patients with a colostomy, many of them still have need for nutritional supplementation and support um and prolonged uh parental nutrition um has been described even with a colostomy. So, um here's just a description um for um creating a potential for um improved um stool outcome or improved um some stool outcomes both in terms of continence as well as in terms of nutrition. Um if the um defunction colon was not removed um initially at birth, but typically, as I said, uh if you, um even if you don't um tize the plates and you decide to leave some of that, um cum on there, you would like to use that hind gut uh and not just leave it blind and attached onto the bladder, especially because of the risk of urinary tract infections. Uh anorectoplasty, as I said, um has been described, uh but obviously, contraindications um are the usual contraindications that we see in Children with anorectal malformations. So, patients who've got severe um spinal defects would not be considered. You don't want to create a peroneal stoma. Uh and you need to work very closely with your neurosurgeons in the majority of these cases. Um They have a significant um incidence of major spinal anomalies. Um And as I said, that increases the risk of enteritis associated with short bowel as well as better to thrive just on its own. Uh The pelvic diastasis in these patients actually causes little morbidity. They tend to waddle, but they often can run and be otherwise quite ambulant if they don't have an underlying um spinal defect, affecting their, their lower limbs. So that was a very quick run through. I've said a lot of uh left a lot of things unsaid. Uh But in short, these patients leave a very heavy healthcare burden. There's a high risk for poor outcomes, a high risk for mortality, particularly with overambitious um surgical procedures. I think the solution to improving outcomes in many cases is delayed surgery stage procedures and very um careful multidisciplinary team as well as um focusing on the small things. Um So nutrition, sepsis, prevention, um attention to detail and careful planning in terms of renal preservation um for patients particularly who have challenge challenges in terms of followup. I think we need to be less medically paternalistic and involve um caregivers in deciding what type of surgery should be done. Um And the timing of that surgery more carefully because uh that's going to improve the the outcome overall for these patients. And we definitely need more uh research into long term outcomes particularly from a low and middle-income country setting. So, uh I'm sure there will be questions, I'm not sure that I can answer them all. But um I would love to, to open the conversation to um to all those who are listening for, for any comments. Uh Th thank you, Marion. That was a nice overview of a very complex uh a quite rare problem uh but which we all uh from time to time are forced to deal with. Um, there is AAA question from Professor Daniel Aaronson, who most of us in South Africa know and thank you Daniel for, for attending. And he's asking, uh, looking at the dismal outlook in the middle income countries, shouldn't the primary approach be palliative, maybe even refrain from sepsis prevention? I think it's a very ethical question. You can answer it. Uh That is a fantastic question. I think it's also important to remember that no palliation basically means the relief of suffering and, and, and that's, that isn't um synonymous with just, uh, you know, pain relief and sending the pain home, the patient at home, um that can involve some surgery. Um So for example, um you might decide just to bring out an ileostomy to make care of the patient easier, particularly from a skin protection point of view. Um And also just sometimes good for the patients, the parents to feel that you're doing something for the patient, but then you might leave all the rest of it and just be conservative with your management of the omphalocele, et cetera, et cetera. Um II, think um the prognosis for every patient needs to be individualized depending on what their spine situation looks like and what their social circumstances look like. Um, we've certainly, um, you know, had, had patients who've pulled through remarkably and I think the literature does show that with good support. Um long term, some of these patients actually, you know, develop a very resilient attitude. Um, and, and can actually do well. Um, I mean, I think of one patient of ours who, who was an absolute bombshell above the belt, but it probably disaster below the belt. And um, if you've got uh a family who's motivated, who, who's um, is able to walk the walk, um I think we are quite conservative. Um, where, where I am in terms of um considering patients for, for pull throughs, for example, but um many patients with adequate nutritional support and I II really want to emphasize um sodium supplementation can actually do well. Um even even though they may have unreconstructed um bladders um and are incontinent that point of view, um The those things can also be managed later on if the patients prove themselves in the um sort of first few years of life, um then you can relook um at managing those, those issues much, much later depending on how, how, how they do. So I think um palliation in some is something that we're very familiar with in low middle and in um income settings, it's something we do uh to a relative degree pretty much every day. Um And I think there are degrees of that. Um I think yes, there are cases where we certainly from the get go have a um a multidisciplinary meeting and say that um we are, we are not going to provide any surgical interventions and, and many of those patients have significant other associated abnormalities and, and pass away quite quickly. Um But I think one needs to be sensitive to the um to the the cultural context as well for that. Um And I think uh palliative surgery is an entity which um which is um not always well understood, I think, but there are many of the procedures that we do, for example, Stone can be seen as palliative procedures in that we are providing relief from suffering, but we're not necessarily committing to doing more than that. I don't know if there are any other comments regarding that um from other people. All right. Yes. If anybody else has a comment on this ethical dilemma, uh please unmute yourself and make a comment while we are waiting for that. Uh I think uh that's an ethical issue but uh it can be said as it's like a Coral regulation syndrome. So below the bed, the child is not well, but above the bed, unless there are lethal cardiac abnormalities or severe neurological abnormalities with uh hi hydreia or something like that. I think we cannot just abandon these Children and uh, you have correctly uh pointed out and advice less is better. So the minimum surgery and we also follow the same uh in East Slender now that we don't close the bladder, we just bring the two bladder hearts together and try and see if we can do an end colostomy. But uh I'm very happy to know about this rescue procedure of end ileostomy in the beginning before doing the end colostomy later and, and not using that uh hind gut for anything else. And uh we have made those mistakes, Marion uh in the beginning of our practice in late 19 nineties where we also took out the testicles and, and tried to rear a child who was born as a boy as a rare as a girl. But at 4 to 5 years of age, the child came back and the granny said, doesn't want to play with dolls, he want to play with boys. So those mistakes are to be prevented. And I think we should not go against nature. What nature is given by chromosomes and by internal and external organs, we should just try and help and not just go against it. And so uh I see there are colleagues from rest of the country. I see uh Professor Tip is here and your experienced from SMU and now in Limpopo or Mpumalanga, you can unmute and, and comment. Sure whether um yes, go ahead, please. Yeah. II yeah, really a very nice uh presentation. I missed a part of it. I was just finishing in theater, but uh we don't have experience with this. And uh I think some of our practice are really uh outdated and uh I really learned a lot from this. So I think it's just going to change how we look at it. But II also support, um, the issue, the issue of, um, uh, individualized in this case, not really p all of them, some of them, uh, they fight for themselves and then we really, our support and we can see as far as we can go. Um, but I think, uh, with the interest from, uh, doctor, uh, we're going to maybe improve our practice and uh have a much better service on this. Thank you. II think, you know, the reality is that, um most of us, I think with this condition can, you know, more disasters to share than, than, than not. But I think that we need to learn from those cases. II think, you know, when things don't go well, when we lose patients, um or when we have suboptimal outcomes, then we need to, we need to actually put that information together, share that information. Um and, and, and learn and grow from it. And I, and I think there is uh even in, in, you know, um high income countries, there, there are, there, there's not a, you know, there's sort of textbooks which say the one thing, but it's the same authors saying those things. And I, and I think we, we need to um sometimes take a step back and, and see, you know, can we do something differently to reduce the number of overall procedures that these Children go undergo? I mean, it causes major anxiety for these patients long term. Um All these um sort of things um really affect quality of life and, and having a sort of overall bird's eye view um which would be ideal just to find ways that we can reduce the number of interventions that also optimize the the outcomes for these patients overall. And sometimes um not not aiming for the the the top, but aiming for somewhere in the middle um might might allow us to, to achieve that. I think maybe it might be a very uh useful information to collect uh information from different centers in South Africa. About, let's say their experience of last five years of management of clinical dystrophy. And what were the challenges? Uh uh what, what could they do? What could they not do? And I think that will be something very worth pursuing. Um Next, I'm going to ask doctor Andrea KK who trained in Durban and practices in Pietermaritzburg. So Andrea Durban experience, Marburg experience. Um Hi, I I've actually relocated, I've joined uh pro pillar uh in Mango. But so from mango side, we I've only been here for, for four months. But yeah, like you mentioned, we haven't seen any since I've been here and then in Maritzburg. Um also you don't know if someone else is present that would like to come in from Maritzburg side, but I can't remember us, seen one there uh in Durban. Yeah, it was al also maybe one or two in my four years of reach time. Um And yeah, it's just a very challenging thing to manage. Um But yeah, thanks for the presentation. Um Yeah, II looked at uh when I was going through the literature, there was a, there's an application from SMA um based in Durban, I think 2018. Um we reported just on bladder ECs outcomes, not so much on, on tobacco ECs outcomes and um 40% mortality. So it's quite sobering um to, to, you know, sort of often focus very much on what happens in the neonatal period because the appearance of this has got, you know, such a unique appearance and it sort of sticks in your memory. But I think one needs to look um really look at the, at the long term outcome for these, for these patients as well. I'm not sure if there is any colleague from Peter Maritzburg. Uh If there is one, please just unmute yourself and, and give your comments. If not, I'll just ask Doctor Mala who trained with us in East London and has been practicing in Port Elizabeth for the last couple of years and he has special interest in urology. So, Doctor Mala, any comments? Uh Good day p um Thanks Mary for the talk. Yeah, it's a very challenging um pathology to manage surgically. Um I if I can remember, I think we had one or two in East London when I was still training and also it was just a very difficult um patient to manage from uh firstly, just trying to get the pelvis to close and trying to formulate the urogenital and the pine gut system to try and separate those areas. Then it it's also infection uh especially in the neonatal period that you you struggle with. And then also just because it's such a complex um pathology that there's also other complex uh conditions. So it's also a matter of um how well the patient is looked after between the, the surgeons and the pediatricians to have a better outcome. If I'm, I'm not mistaken, I think one of them that we did last prof um the patient did go do well and was discharged and we were just managing the, the problem of um obviously having an a an end colostomy inverted commas and then there's just struggling with the urine cause of a small bladder leaking all the time. We have one currently in our unit, which we closed. Uh We tried to close um a week ago, but we struggled because that pelvic space was so small. We couldn't get the two pubic bones together so that it's still sort of like an open wound type of thing that we're trying to manage with conservatively with dressings. Um We couldn't get a proper sort of um urethral opening over the catheter. So we've, we've got um, and open bladder still on that patient. So it's still complex and still uh a difficult case that we're still trying to uh manage. And basically, the main thing is to just make sure that the patient can be discharged with some form of um passage with an ileostomy and some form of output from a the urogenital point of view. Yeah, thanks for that. Um Gko, I think, you know, I think there is definitely a push um especially overseas for, for um some isolated centers or selected centers if I can put that way. Um just to try and increase the, the volume of those a few centers. Um So, so that the overall experience for these patients can be improved. Um And I, and I think that is something that we should also be looking at in South Africa to say, OK, well, you know, the center is going to look at that area, you know, manic from that area, et cetera, et cetera. Um because it's, you know, these patients are very, very challenging to, to manage. Um And I think, you know, the the complication is high in, in the best of, in the best of hands. Um So, you know, challenges with, with the failed um data repair, I think from what I'm understanding is from the literature, let it be for at least 4 to 6 months. Um you know, consider sending it to, to pro Lazarus um to do the next stage and definitely look at getting osteotomies to, to, to for the next attempt of, of closure, what, um, you know, whatever that may may involve. Um, but in the meantime, you know, just look after the kidneys with continued, um, antibiotics, prophylaxis for that. Um, and obviously look after the child from a nutritional point of view and an overall point of view and, and also, you know, don't forget about the spine um in terms of working the child up and, and, and managing that aspect of it because the, the this um it was interesting to me that to what extent the the spine actually plays a role in terms of overall prognosis. Um you know, in that, that patients who have got major spinal defects have worse outcome from a, from a bowel point of view as well as from a bladder point of view, as well as the ambulation and quality of life point of view. Yes. Uh Thank you, Veronica. I think Marion, we consider it an achievement if the child survives neonatal period in early infancy. And over the last 30 years, you probably have had six or eight clinical trophies. And uh we remember closing the bladders doing in colostomies, but we don't remember those patients coming back for us to consider bladder augmentations or bladder neck closure, et cetera. So we don't know what happens to those patients, whether they just die and we don't have mechanism to keep track of them. So, the last uh consultant I would like to ask uh comment to comment is Doctor Selo Vaya, who is one of our senior consultant, pediatric surgeons also has uh interest. So sell your comments. Thank, thank, thanks very for the talk. I think um I'll just echo what everyone is saying to you that it is a complex uh condition to manage. But what we have learned recently is what is saying that um so that letter should not necessarily be so high up on the list because that's where all of the issues come. We're very well and able to if you can from this. But trying to actually um bring the ladder two houses, the bladder together, close the ladder approx the s that is just a big picture II believe. And with what a lot of um what what people are saying now they the entire can not continue to use. The only try that you can divert to do it. Do you think you're a little bit there? Just a little bit closer to the Sorry, I start to hear what you're saying. Yeah, that's better. Yeah. Yeah, sorry. So the the main thing is just ok, try to get that. There's a lot of for you still have to deal with that. Um And sorry, it might be my internet. Uh You are hardly audible if possible, maybe just type your comment into the chat box. I'm sorry. Yeah, I'm I'm sorry. We couldn't hear Doctor Mach's comments. It could be my internet. Uh Maybe he's got a message here. Uh I know he's also, it could be the internet from his side. So, Marion, I think uh your concluding message. Thank you for the talk and your take home message. Thank you very much. Yes, I mean, I think it's not all doom and gloom. Um We have got a patient from East London currently in our ward um who is undergoing further urological reconstruction. Um And you know, who looks like a normal body until he could take his clothes off, uh probably got a little bit of a waddle. Um But um I think, yeah, you know, it's, it is an elephant of a diagnosis. Um And I think the important thing is to, to attention to detail. So to really take into account the full picture of the patient um from social circumstances through to um neurological status um to, you know, sort of nutritional optimization um to, to sepsis prevention, not being in a, from a surgical point of view to, to go in surgically and to, when you go in to, to go in, aware of your options because you never know what exactly you're going to find the recording has stopped. Um And um I think to, to regularly, you know, sort of review where you are in terms of where you're going with that patient. Um And to, to also consider to, to get help from a friend. Um So, uh these things are challenging conditions. None of us see a lot of these. Um, and so asking for advice, I think is, is always a, always a good idea. Um, and, yeah, the, I think, um, my, um, sanitation to, to the parents, um, and the patients themselves who we've seen over the years to, uh, who've taught us a lot. Um, and, um, certainly the, the ones who have had pulled through, um, have often done remarkably so under, you know, with challenging conditions. Um, and some of them, we managed to get, you know, clean and dry even if it's with the two bags. Um, but some of them are doing ok with, with that. Um, and I think we just need to, to bear that in mind. It is possible. Um, but it takes a big team effort. All right. Thank you very much. Um, thanks for an interesting topic. Um, me, thanks and bye-bye, this meeting is being recorded. I, I'm very sorry. Uh, we have this curse called load shedding here and despite of me having a battery backup, somehow loaded.