"Duodenal and small bowel atresia" by Dr Chrystal Johnson and expert comments by Prof Sherif Emil, Montreal, Canada.
"Duodenal and small bowel atresia" by Dr Chrystal Johnson and expert comments by Prof Sherif Emil, Montreal, Canada
Summary
Delve into a comprehensive analysis of duodenal atresia, as well as ju atresia and stenosis in this on-demand teaching session. Understand the congenital abnormalities and defects implicated in these conditions, and learn about the intrinsic and extrinsic causes related to developmental challenges. Unpack how it occurs embryologically, from the moment the duodenum begins to develop. Discover the links between these conditions and other abnormalities, such as Trisomy 21 and congenital heart defects. Learn how to diagnose these conditions antenatally or postnatally and explore various management and operative strategies for these abnormalities. This session is highly recommended for medical professionals eager to expand their knowledge on these critical medical conditions.
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Learning objectives
- Understand the etiology and clinical manifestations of duodenal atresia, ju atresia and stenosis in the context of pediatric patients.
- Be able to recognize and interpret relevant diagnostic imaging for duodenal atresia, such as the classic "double bubble" sign on plain film and ultrasound.
- Learn to identify potential complications associated with duodenal atresia, ju atresia, and stenosis, such as associated congenital abnormalities, intrauterine growth restriction, and electrolyte imbalances.
- Become familiar with different surgical interventions and procedures for managing duodenal atresia, ju atresia and stenosis, along with their associated risks and potential complications.
- Understand the prognosis and long-term outcomes for infants affected by duodenal atresia, ju atresia and stenosis, including post-operative care and potential late-onset complications.
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Points of discussion originally were pyloric stenosis. But we'll discuss that at a later stage. Duodenal atresia, ju atresia and stenosis will be discussed for today. And colon atresia, we'll also um discuss at a later stage. OK. So with regards to duodenal atresia, it's a congenital absence or clo or complete closure of a portion of the lumen of the duodenum due to defective fusion of the for gu and the midgut. With failure of recanalization, it occurs in one in 5000 to 10,000 live births and males seem to be more affected than females. Um with regards to how it occurs. As I've said, there's failure of recanalization which is an intrinsic cause of this problem. There are also extrinsic causes associated with this defective development of the adjacent structures. And other teratogens have also been implicated in in this with regards to intrinsic lesions. The failure of recanalization which occurs obviously in the embryological stage, the fourth week of gestation. If we just focus on the picture on the left, we can see that the duodena begins to develop around the fourth week of gestation and that is from the distal foregut and the proximal mid gut. Um during the 5th and 6th weeks of gestation, duodenal loom and then temporarily obliterates um completely because of the proliferation of epithelial cells. There's vacuolation which occurs um and degeneration of these cells happens around the 11th week which then results in a recanalized um duodenum. The embryological insult in this period is thought to what leads to their intrinsic webs, atresias or stenoses with regards to the extrinsic form or the picture on the left is just one example of it. And that would be your annular pancreas. You could also get a preduodenal portal vein or a secondary to malrotation which leads to your la la and la veins with the annular pancreas. Um This does warrant special mention because there's a form of obstruction which is likely also due to an intrinsic duo duodenal developmental problem as well as the external compression. So the angular um annular pancreas is a visible indicator that there is an underlying stenosis or atresia present again between the fourth to the eighth week of gestation is when your pancreatic bugs merge and the tip of the ventral pancreas um seems to become fixed to the duodenal wall which forms a non disable ring around that portion of the duodenum um in the descending part of the duodenum. And then the annular pancreas is also associated with duodenal obstruction. As I said, the biliary tree can also be affected and that can be to the proximal or distal part um of that as well. There's several via varieties of intrinsic and extrinsic lesions as we discussed. And 81% of those will cause complete obstruction. While about 20% of that causes partial obstruction to the duodenum. Then more than 50% of the patients affected by duodenal atresia also have other associated congenital abnormalities. Up to a third have trisomy, 21 a third will have isolated cardiac defects and a quarter will have other gastrointestinal issues as well. 45% of the patient population will be premature with a third of them exhibiting intrauterine growth restriction as well. And as I've mentioned, the associations, they can be associated with down syndrome, Angula, annular pancreas. Then the the third with congenital, also malrotation is something to think about. And then you've mentioned the multiple other atresias. Then with regards to the classification, you'll follow the gray and scan. The lar um classification which divides it into three types, type one, which is the majority of the cases, more than 90% of cases present with this. And there's an obstructing septum or web formed from the mucosal and submucosal, but with no obvious muscularis defect, noted the mesentery is also intact with this. Um the one thing with this that we need to be aware of is the what's called the windsock deformity. And that is when the membrane is quite thin and elongated and seems to bellow from the second portion of the duodenum, right into the 3rd and 4th portions of the duodenum. Um The septum itself that forms in the second part can be of varying thickness from a couple of millimeters to several to several millimeters. Um It can be an imperforate septum which will then cause an complete obstruction or have a central perforation which will cause an incomplete obstruction. And then the diameter of this opening will then obviously inversely um be the degree of your obstruction. And then with regards to the intrinsic blockage, this can happen in almost any part of duodenum. But like I said, more commonly, in 85% of cases happening in the 1st and 2nd part of the duodenum. With regards to the intrinsic um intrinsic obstruction, particularly with angular pancreas. There is this ring of pancreatic tissue which is encircling itself around the duodenum and causes partial obstruction. But like we said with that, there's often associated underlying duodenal atresia and developmental problems um with the recanalization, a preportal duodenal preduodenal portal vein. My apologies can also then cross the anterior surface of the duodenum rather than posterior um in its normal anatomical association and may cause incomplete obstruction or compression of this as well. It can also be intestinal malrotation and which results in lands lands bands also again causing obstruction more in the 2nd and 3rd portion of your, of your duodenum. And many of these patients with extrinsic causes are the patients with the cardiac cardiac lesions, as mentioned, then moving on to presentation and diagnosis, the benefit to antenatal diagnosis um is that you can have parent counseling and that also further investigations can be done for associated anomalies. And some of the antenatal features that can be found is polyhydramnios seen. And if fetal ultrasounds are done, a double bubble will be noted. And that's often in late gestation only in the seventh or eighth month of gestation is that seen at that point. One can also recognize an annular pancreas. Um I must say in South Africa, I think our antenatal care is is not on par and we often don't have them diagnosed antenatally in our settings. Unfortunately, then with regards to the neonatal period, an actual clinical presentation, repeated bilious emesis or vomiting is what can be noted. There's also minimal distension because the obstruction is so proximal. It's often just a very subtle upper upper abdominal fullness that is noted. Um There may be a report of poor passing of stool or flatus. Also feeding intolerance is another one in the baby because of this ongoing vomiting may present with the dehydration and electrolyte abnormalities and moving on to further investigation. If we look at the plain film, I now mention the double bubble with regards to an ultrasound done antenatally, but post postnatally, this is what the classical sign um is referred to as where you can see a distinct stomach bubble and a distinct bubble of the duodenum and a pretty much gasless um abdomen in the rest of the distal bone with regards to further investigation. If you're still unsure if the settles are, if the signs are quite subtle, especially if the ampulla of vata is distal to where your obstruction is that you may not get the bilious emesis. Um and that's in 10% of cases, one can then do a upper gi contrast study as seen there, um which will also then um nicely outline where the problem may lie with regards to the management placement of an oral or nasogastric tube to aid the decompression. Um fluid resuscitation is important and to correct those electrolyte abnormalities with regards to the blood work, that's important. A full blood count. A CBC is important uh urea and electrolyte panel as well as uh calcium magnesium and phosphate profile and, and, and, and a coagulation studies are important as your preoperative workup work up also. Um and an electrocardiograph is important because of those associated cardiac abnormalities. And we look for other structural abnormalities and intraabdominal issues with an abdominal and spinal ultrasound. If there's any concern that this is a foregut volvulus surgery at that point is, is quite emergent. We're looking at the operative management options. We can either do it via open procedure and do a laparotomy by utilizing uh um super supraumbilical incision, transverse incision at least or it can be done laparoscopically. And it's the following uh methods are amenable by both approaches OK, one needs to adequately mobilize and expose the duodenum so that you can see the extent of the problem. So you need to mobilize your colon inferiorly, you also need to then cauterize your duodenum adequately so that you can see the full extent of the problem. In which case, it may be necessary to taper it to decrease the dilatation. Um The other option, if you're going to excise a web, in the case of a type one, we can do a high duodenostomy. Um And then there are other options in terms of bypassing that. And a duodenoduodenostomy using a diamond shaped configuration is another approach that one could use. The duodenojejunostomy is also another way to overcome very tricky space to work in. And you can kind of sometimes aid yourself by doing that approach, although it runs the risk of a blind loop syndrome and then the less favorable and often not used now is the Gastrojejunostomy and that's because of marginal bleeding and the risk um of ulceration from that particular procedure. With regards to the complications intraoperatively, you may not correctly identify the area of obstruction. As I said that windsock deformity can be quite tricky as the thin membrane beds quite, quite far distally. And you may not actually notice that the one way to overcome this is to pass a catheter both distally to ensure that there are no additional webs or or areas of stenosis or atresia. And then also to pass a catheter super even into the stomach to make sure that there's adequate, um there will be able, adequate drainage from the stomach and there's no obstructing web, more proximal than you also than you thought to put your anastomosis. Um and thereby mitigating the m the most distal web as well by doing that postoperatively. If the infant is then taking a very long time to feed more than three weeks, one can consider an upper gi series and also to delineate any residual anatomic obstruction to see if there's any anos anastomotic stenosis. Um Also any previously unrecognized obstruction or just to see what the peristalsis activity of that particular section of bowel is as well. Some late onset complications is a partial web, narrowing of your anastomosis and stricturing over time, poor peristalsis and further dilatation because of obstruction. Um And that can also be dealt with with any of the surgical procedures described previously um as well as pre prescribe quickly with outcomes of um any of these of the procedures described and operating on these babies of up to 95% survival. Ok. In the gal ideal atresias, they occur in one in up to 5000 births and therefore, are more common. About a third of them are associated with prematurity and being small for gestational age. Males are as affected as females with regards to how this occurs. Um The vascular disruption in fetal life has been the proposed and accepted um theory based on studies done um previously and it's um localized into uterine vascular insult which will to the developing bowel, which causes ischemic necrosis and subsequent resorption of the affected segment of bowel. Um Sometimes the additional pathology that actually supports this is because there can be inter intrauterine fetal interception malrotation with a midgut volvulus. There can be thromboembolic occlusions, trans mesenteric, internal herniation and incarceration or a sneering of the fetal bowel in the gastroschisis or omphalocele, which supports this hypothesis of um ischemia. Then what adversely the ischemia adversely influences the structure and the subsequently forming of the remaining bowel histologically and histochemically, there are abnormalities and that can even extend as high up as 20 centimeters from your point um of atresia, your proximal segment of your atresia and then the distal bowel is potentially normal functioning bowel. There has been um a a proposed genetic or familial, although the genetic contribution of a atresia is unclear, shorter. And his colleagues came up with a novel system whereby they looked at the patterns of inheritance with regards to the different types of atresias. And they've come up with their own classification. Although, although it's not, not in much use of, of of much clinical significance, it is interesting to note then the classification that we are going to chat about would be your Grabel classification, which looks at um stenosis as well as other pure types of atresias. The stenosis is defined as just a local narrowing in the intestinal lumen without any disruption of your, of your intestinal wall or your mesentary itself. And when we look at type one, it may sound that type one can also take, it can take the form of type one by just having a fenestrated membrane as well. And patients with rein stenosis usually have normal length of bile. When looking at type one, we can see there that it occurs um secondary to again a membrane or a web forming within it encompassing the submucosa and the mucosal layer with an intact muscularis layer. And on growth inspection, it may look as though there is bowel continuity. Definitely it looks like that with mesenteric continuity as well. But the proximal bowel is often dilated. While the distal bowel, as you can see in the picture, um looks quite collapsed and a lot more attenuated with increased Luminal pressure. That proximal portion starts to dilate a little bit and a bulging web may also cause that windsock effect like we discussed in the duodenal atresia. And we just need to be aware of that as well. With this, there's also no significant shortening of the bowel itself with regards to the type two atresia. It's a largely um there's a largely dilated blind ending proximal segment with a thin, usually thin fibrous cord which is then attached to the more distal and collapsed se section more distally with an intact Mery. Also total bowel length with, with type two is usually preserved to the more serious types. Type three A. We can see that the atresia ends blindly with a non with no fibrous cord connection between the proximal and distal portion. Again, the proximal portion portion seems quite dilated while the distal portion is small is a lot smaller caliber and looks more collapsed. There's also as we can see a mesentery defect in this, usually the total length is slightly less than normal. And that's owing to those vascular accidents where there's been inter uterine resorption of the of the, of the bowel in in between those sections. Then the type three B atresia known as your apple peel or Christmas tree type configuration. And there's it's a very proximal genital atresia with the absence of the superior mesenteric artery beyond its origin um of the middle colic branch. Also agenesis of the dorsal mesentery is present with significant loss of intestinal length and a large mesenteric defect as can be seen in the picture. The in the distal bowel is quite decompressed and lies free in the abdomen and also takes on this helical um configuration, which is where it gets the, the name characteristic of an apple once it's peeled. Um with regards to this again, it has been found in families and like I said, the genetic pattern for type three B has been attributed to autosomal recessive mode of transmission, seen quite significantly between siblings where they have identical lesions and in twins as well. There can be associations with o other abnormalities with type three B. Significantly more. About half of the presentations are accompanied by a male rotation. There can also be short bowel syndrome in up to 75% of the cases purely on the native bowel without resection even and then carries with it a higher morbidity rate of 63% and a mortality rate of just over 50% of the affected population. Then with regards to type four atresia, this is multiple segmental atresias or a combination of one through three. And that has been grouped together as a type four. And to date, a type four has been almost uniformly fatal and doesn't have um a good, good prognosis at all. The majority of patients with multiple segmental atresias are sporadic and there's no family history of gastrointestinal defects often with this and then a differential diagnosis for what we're seeing is can be a malrotation with or without a volvulus. If there's a meconium ileus, um if there's distal intestinal duplication, sorry, any internal herniation, if there's colonic atresia, a paralytic ileus or total colonic, a ganglionosis can also present the same way as well as an infant with hyperthyroidism or um a another on medication that causes a similar problem, then the clinical presentation again, antenatal diagnosis, um or clues are there such as polyhydramnios and then if you can get a fetal ultrasound later on, you'll know, dilated loops of bowel with presentation within the neonatal period can happen anywhere between 1 to 3 days depending on the level of obstruction. And then the baby will present with obstructive symptoms being the bilious emesis. The di the distension on this baby is more significant of something very distal rather than just a gal eye. So maybe misrepresenting the abdominal distension a little bit on that. But that is another finding. Um And yes. So then with regards to the bowel obstruction noted by the previous emesis and the distension, there may be meconium and that may actually appear normal or can look like little gray plugs plugs um that are passed per rectum and often that can be a little bit disconcerting as to why is the baby passing, passing meconium? But there's such gross features of obstruction with regards um to the management. Again, these are neonates. So we want to look after them as best as we can maintaining the heat, always put in a or or a gastric tube preferably size fringe. But if you have a tiny neonate, then obviously a more appropriate size to them and also to aspirate whatever comes back just to see the color and the volume of aspiration and intravenous access is important whether it be via central line or peripheral line initially for fluid resuscitation and maybe later on for parenteral nutrition to consider something more long term and central access blood work up as for duodenal atresia again to do a full blood count to you. And ECM P coagulation profile um is also important, monitoring their inputs and outputs because they are prone to dehydration and third space losses. Um And then also antibiotics, especially when you have an impression of perforation or there's other concomitant infection that's important in operative management. If we're going to go surgical, if there's any indication of perforation, then one would advance to surgical management quite quickly. Um Even if the diagnosis is not completely confirmed, but that would then Trump, you need to operate. If you're going to do a trans uh traditional transverse incision, it can be supra or infra infraumbilical. Um the step towards doing an umbilical incision where you just vertically, then incise the sheath to create some adequate exposure for yourself or a laparoscopic approach where you start laparoscopically and identify, identify the atretic portions and then extracorporeally fix those segments is also being done and often easier to do it extracorporeally. Because if it is a tiny neonate, there's very little space in which to work. Some of the surgical considerations is finding the location of the lesions and any associated conditions as I've explained earlier, also the anatomical findings and then determining patency distally which one can do intraoperatively again by passing a small feeding tube and with instilling saline into the bowel, noting where it dilates and also whether it passes rectally, then also the length of remaining bowel is important to measure um especially when tapering and also trying to limit your resection so that you maintain that as best as you can. So then your location of your lesion, this particularly picks uh type three but a three a at least. But, and when you do your resection to resect a little bit more proximally on the proximal end and a little bit more distally because as we said, those functional portions can extend quite far back. Then one can do um into oblique um anastomosis of those. In order to gain diameter on your distal portion, you can also taper those portions so that you your calibration between the proximal and distal portions are more or less even when looking at the length. This is measured on the anti mesenteric order and the length of more than 80 centimeters with a competent and intact iosal valve is an ideal ideal scenario, but it's not always possible. There are um length gaining procedures, but they are often not the first choice to do when operating at the initial operation. When tapering, you can taper for up to a length of 35 centimeters. And that's being quite generous. You can either do a hand. So um tapering method or you can use a G A gi A stapler as well. In order to do that, you can also use application method where just um cirrhosal layers are incised and then uh and then approximated sorry with the unfolding portion can be quite long but not more than half of the diameter. And the same with the folding method, you don't want to go too deep and thereby obliterating and blocking, blocking your, the lumen that you have created with regards to postoperative complications. As was the duodenal atresia as well. Infection is always um one of the things leaking from your anastomosis, like there's any obstruction, not previously noted or stricturing down of your anastomosis if you've done more than one, and even one and then the significant complication which is evident later on would be your short bowel syndrome. Immediate postoperative care is concentrated on providing parenteral nutrition by, by um central line, maintaining the fluid and electrolyte balance and keeping a close eye on that. And then also initiating of enteral feeds. Once your aspirates are quite low, there's no absolute number. And this differs from institution and surgeon and um medical team involved. But once aspirates are clear and minimal, then one can initiate enteral feeds also in a stepwise approach as per institution. This is more for interest sake for bowel lengthening procedures. Like I said, like I said, are not advised at your initial surgery, but there's the longitudinal intestinal lengthening and um tailoring procedure or otherwise known as the L which does gain you length, I think um technically, quite difficult and does place a lot of pressure on the reason tree when doing it. Then you get the serial transverse enteroplasty also, thereby gaining length by changing the configuration of the muscle fiber orientation and a slit procedure. The colonic atresia we won't go through at this point and that concludes the talk. Thank you OK. Crystal. Thank you very much uh for keeping to time and uh just for time constraint, we decided not to include pyloric atresia and colonic atresia. Um um crystal. There will be lots of advice by the seniors. I just wanted to um mentioned that we have not done uh Heineken Mikulic duodenoplasty or Duodenojejunostomy for Duodenal arteria. I can't remember. So I would like to know from, from Doctor Emil and uh pro if they have ever done it. That's number one and about small Bile Atresia. I'm sure you know, uh and we all South Africans are very proud that the etiology was discussed uh described by Professor Low and Doctor Christian Bernard when they did those uh experiments in the puppies and uh just type for uh Atras II heard you saying that they are uniformly fatal. I think that's not the case. Um depends on, on the length of bowel. Uh So, so that's, that's not the case and use of transanastomotic uh tubes for feeding. Uh We will leave it for the later discussion. So, um I will now request Doctor Emil uh to give his opinion, present his, his views on on this topic. Sheriff, my apologies if I did not uh clarify that was I think I caused a little confusion. Um I thought it was only small bowel Atresia but uh, uh Crystal has also spoken about Duodenal Atresia, but you can, well, yeah, I mean the Duodenum is small bowel so I can't fault her. Sorry. Ok, you can go ahead. Thank you. No, I just wanna thank you again. Uh, I wanna thank Professor Chits again for the invitation and I see Professor Hadley there also on the call. Yeah, and Larry was the first uh South African pediatric surgeon. I met, we met on a flight on the way to a paps a meeting and I have to say uh I maybe never thank you, Larry. I'm sorry. But II owe part of my position here at mcgill to him because he was one of the external reviewers for my promotion. So uh I II think he gave a good review otherwise I wouldn't have been in this position. So thank you, Larry many years later. Um And Crystal, that was a very excellent review. II just have to share something with you. I was laughing as I saw your background because I was in Africa when COVID struck last March and I had to, I'm back in a hurry from Senegal and I was in quarantine and our hospital had, had just bought zoom. And so they were teaching us how to use zoom and the instructor had this background and it was mid March, it was very cold. We had a lot of snow and I kept asking him, where are you doing this from? Where are you doing this from? And he never answered. And I thought gosh, that's really rude. Why can't he tell me where he is? So I learned a lot about zoom since later on much with the background to change it. So I apologize if it was distracting. Yeah, be beautiful background. I did focus my comments on small bowel and colon, but I do want to just make a few points about um the duodenum. So annular pancreas, first of all, that was actually very fascinating because if you look at the history and I'm also a bit of a history buff. I like to look at the old papers. Um you know, surgeons for decades uh in the early part of the 20th century up to the fifties were removing the annulus. And then, and of course, many of these patients will die from pancreatic leaks. But they were also wondering why is not the uh you know, their obstruction resolving. And we've really learned that there's probably not an extrinsic compression issue at all. It is really the internal atresia or stenosis that accounts for it. Uh So it's really a marker for a duodenal intrinsic problem. Um The other thing you mentioned is uh when to do a contrast study, when you have a double bubble. And that's a very important point because, you know, as surgeons, we do not like complete obstructions, right? We like partial obstructions more than complete. But the the only exception and is a duodenal obstruction. When you have a partial duodenal obstruction, I ea double bubble with some distal air, you have to entertain the possibility of a got valvula and that's the patient who should get either immediate exploration or an immediate contrast study if you have a simple double bubble, especially if it was diagnosed prenatally, but even not. But if you have a double bubble in a stable patient, no distal air, that's either going to be annual pancreas or duodenal atresia. There's really no indication for doing in my mind, a contrast study on those patients. Um The other thing that I did want to mention also is the issue of TPN. I think uh you, you, you, you mentioned it earlier. So for duodenal atresia, we typically put transanastomotic tubes. And in fact, most of these patients do not get a pick or TPN at all because they usually use a transanastomotic feeding until we're ready to feed them by mouth. For duodenal stenosis where there's a diaphragm Windsock uh web, whatever you want to call it. I do not do an anastomosis. I do not do a bypass. I always do a duodenoplasty and it's really not so much about excising the web. Uh When you open the duodenum laterally, you're ready to drop the web enough. And in fact, I don't try to do much to the web because often the ampulla is coming right at the same point. So I might excise, if there's really a true Windsock, I might excise part of it distally and there's a, uh, some beautiful pictures of Windsock in my book. But it, I really just do a duodenoplasty and II have to say maybe this is my bias, but I don't find a huge need for laparoscopy in most of these patients. I mean, they can be done through a very small cosmetic. Uh It, it is minimally invasive surgery still, but it's not laparoscopic. So II, you know, with, with all due respect to the people who use laparoscopy for many of these, II don't find really much use. So I'm just gonna go through uh I think you all know this, it does help very much to, to speak about where the obstruction is. Uh when we're talking about obstruction, this is a paper I'm going to share with you after the talk, I'll send uh me some papers uh to share with the group. But this is a paper from doctor rickets almost uh maybe a quarter century ago or more. And even though the timing of intervention has changed for some of these, the work up is really pretty much the same. And it's just a great synopsis on uh the workup of a new na intestinal obstruction. I always share this very early with our fellows when they come and start pediatric surgery. So here you have two babies and they're both having, they're both transferred in maybe on the first day of life with bilious emesis. And you see one on the left that's quite distended and has these corresponding films and you see one on the right that is really scaphoid and has these films. And of course, the next point is what do you do with these? And, um, because of the time I'm not gonna pause too much. But really the differential diagnosis before you even decide. Ok, what I'm going to do is, you know, in surgery, we think backwards, you know, I mean, the, the the soap mnemonic of doing subjective and then objective and assessment and plan is a great way to learn medicine. But really, if you think about how we practice within a minute or two of seeing a patient, we're already thinking what the diagnosis might be. And then we think backwards to confirm, confirm or not, confirm that diagnosis. So when you see a patient like that and you know that you have obstruction, distal to liris, what is the differential diagnosis? Well, there's not a whole lot, it's intestinal atresia, meconium, ileus, meconium plug or small left colon syndrome and Hirschprung's disease. That's pretty much it. So then what is your next step? The next step is to do your contrast enema. And that's not always intuitive. In fact, until now, we often talk with a radiologist because if they see a proximal obstruction. They want to do an upper gi first. And that does not help. The only time again that a an upper gi helps is if you're suspicious of a mi that values because of a partial duodenal obstruction. So, in the contrast, enema, you go in and, and you really want to ask three questions. Is there a microcolon? Can you reflux back into the distended bowel? In other words, there's no true mechanical obstruction, there's continuity of bowels. So something like a meconium ileus, which you probably don't see in Africa very much. But here in Quebec, we see it very commonly and is the colon patent? Because as you mentioned, you can make a small incision, eviscerate the entire small bowel, but it's not always so easy to explore the colon and confirm patency. And we know that multiple atresias can exist. So if you haven't done that, I like to leave a foley in the rectum and then have the nurse put some uh saline in the bowel. So I can see the colon, the stent and make sure it's patent and that I'm not missing another distal atresia. So here is a study of a typical patient. You can see the distended bowel very nicely and you can see the enema and you can see that the enema refluxes into multiple small bowel loops because most of these babies have an incompetent illo valve, but you do not reflux back into the distended bowel and that's an indirect evidence that you've had a, a small bowel obstruction ie likely an Atresia because you have a microcolon, uh, the colon is patent and you cannot reflux back into the distended loops. So that's really the sign of these types of, of obstructions. You mentioned this already. And I have to say it's, I really approach this with humility, speaking to surgeons in South Africa, because this is very much, uh a South African contribution to pediatric surgery. We've come to understand this uh through the work of law and Bernard of course, and, and, and it just amazes me when I read these original papers. And again, you may have them already, but I will send them you. I actually have the original papers and it is just fascinating, this is, you know, decades before fetal surgery ever even existed or rose to the uh e entered the field of pediatric surgery. And yet they were doing this elegant fetal canine and, and, and check experiments that very nicely showed us uh really that the etiology of Jejunal atresia and and intestinal atresias in general, with the exception of the duodenum. Although I have to say that type one you described is very much like the duodenal atresia rather than the accidental events that occur, which we believe are responsible for the rest of small intestinal and colonic atresias. Now, one of the things I really enjoy about treating this problem is that I don't find that there are two patients who are exactly alike. There's always subtle differences between um between patients. And so when you enter, you really have to have some guiding questions that you ask yourself before you even approach the case. And I like to think of three questions. So the first question is, what is the best option to establish bowel continuity or the conditions for early initiation of feedings? And if you think about each of those questions, they each relate to a specific morbidity. So that question relates to the immediate morbidity, the morbidity of not being able to feed a baby early. The second question is often as you mentioned, uh there can be uh short gut. So if you are dealing and it's not always the case, but if you are dealing with short bowel, what is going to be the best option to preserve bowel length and absorptive capacity? And that, that of course relates to the long term morbidity of short gut. And the final question is again, you mentioned that showed really nice picture of dilated bowel. What is the best option to address grossly dilated bowel? Why? Because that sometimes can be what we call a latent morbidity. And I'll show you a case many, many, many years later and we're seeing more of these patients, often our general surgery colleagues will call us about them now, uh who have problems because they've had dilated bowel that functioned OK for a while. But still comes back to. Now, when you look at these three questions, sometimes you can come up with a plan that meets all three criteria, but sometimes they conflict. For example, you may have very dilated bowel, but you may also have short bowel. So if that's the case, you don't want to be sacrificing absorptive surface early on, you want to maintain all the bowel. So there is where you have to have a plan to give you the best solution that may not be able to be optimal for each of these three questions. And again, the essential operative steps, you mentioned them, you have to confirm the presence and type of atresia exclude po associated anomalies, confirm the patency of the remaining bowel, assess bowel length, establish bowel continuity if possible receptor plicate bowel is appropriate. And, and, and one of the things I'd like to mention earlier is if I'm dealing with one of these cases, and it's evident right away that I'm dealing with short bowel, I like to put a gastrostomy right away because I know that patients will need it. Uh And I don't know in your environment how acceptable gastrostomies are, how easy they are to deal with. But in our environment, I always remind my colleagues do that early rather than having to come back into uh an opera uh uh a case that has already been operated. So I'd like to just go over some cases as you know, I'm, I'm really fond of, of case based, uh learning and teaching. So, uh I'd like to go over some cases and just have you think about them. So, here you have a type four and I agree very much with Professor Chits. You don't always have short bowel. In fact, many times there is reconstituted bowel distally and, and the bowel is fine in length. So here you have a type four, you can see very distended bowel about 20 to 25 centimeters from the ligament, right? So you can see multiple atresias over here and then the bowel reconstitutes and you have plenty of bowel. How would you deal with this? Ok. So here you have to think of the three things. Short bowel is not an issue. So you don't have to worry about preservation of uh of uh a very abnormally dilated bowel. You want to establish continuity and you want to deal with a distended bowel. So one way to go about this is to resect as much of the distended bowel as possible along with the atretic segments. And then usually, if I'm not dealing with short bowel and I have plenty of distal bowel, I'll usually resect to about somewhere between 5 to 10 centimeters of the ligament TRS because obviously I'm not gonna resect right to ligament rights and then whatever I have not resected, I will taper. So here you will see that these are the bowel ends. I then taper, whatever part of the jejunum that is still there. And I do an end to end anastomosis. And again, here, it's not about preserving bowel length. So you fulfilled your goals by dealing with the plaque with the distended bowel, establishing bowel continuity right away and initiating feedings. Hopefully soon after. OK. Here you have a new needle bowel obstruction. OK? And you have a very, almost like what I would call a triple bubble if you will. So you know that this is a very proximal obstruction. Now, you've done an enema here and the enema is showing you a microcolon. OK. Uh Except for the rectum, of course, but it's showing you am an unused colon. Now, what do you worry about going into this scenario? You have a very proximal obstruction and a microcolon. Should this be expected? And the question is when you see this scenario, you have to suspect that you're gonna deal with a case of severe short bowel syndrome. Why? Because with very proximal Jejunal res uh just like duodenal res, if you have the bowel normal past that you shouldn't really have an unused colon because uh there's a lot of desquamation and so on. Really most of the elements of the meconium, with the exception of the bile should have passed through the distal bowel and you should not have the typical microcolon. So the more proximal, the obstruction, if everything else is normal, the less likely you are to get a microcolon. So when you have a proximal obstruction like this, plus a micro colon, you're gonna go and operate on this patient expecting that this is likely going to be a challenging short bowel syndrome. And in fact, this is the patient uh not so long ago and you can see there's about a 15 centimeter uh distended jejunal segment, but the, the maximal distension is about three centimeters. So, not, not massively massively distended. And then you have a distal ileum about 3 to 4 centimeters. And this, the colon and appendix are normal and this is a case of atresia most likely due to a midgut valve or significant compromise of the entire bowel segment. So, what do you do in this case? Now, here again, you look at the three issues. Am I going to deal with this and plicate it or remove bowel or even invert it or injure it in any way? No, I do not want to do that. I do want to establish bowel continuity if possible. And short bowel is going to be an issue. So in this case, I just did a primary anastomosis, a primary anastomosis, accepting that I'm going to leave this bowel behind, but that I'm going to and I put a gastrostomy that I'm going to be able to feed the baby at least, uh uh uh some amount of feeds early on. And what happened here is that this is the anastomosis gastrostomy and then on this patient later on, we came and did a step procedure and amazingly because she had bowel continuity early and the bowel wasn't severely distended. We were actually able to feed her within about two weeks of, of doing all this. And this baby never developed TPN cholestasis because she, oh and this was actually before we had omegaven and, and you know, liver preserving, liver protecting TPN. But this baby actually did very, very well and never developed TPN cholestasis did go home on home TPN but was weaned off it around two years of age. Ok. Here again, you have a new needle bowel obstruction, again, very proximal. And once again, here you've got a, a colon that looks like an unused colon. And again, you have to suspect that this is going to be bad and you go in and you find this problem. So here, now you have a severe inflammatory condition. Um, this picture doesn't do it justice. But what I found here was a very distended jejunum and a big Phlegmon, a severe inflammatory mass. I could not tell what's what I couldn't see any distal bowel. And it was very clear that if I persisted, I'm going to cause very significant injury to any bowel that may still be there. So this was actually before we had access to a small and omegaven these liver protecting TPN formulas. And in this case, I wanted to be able to trickle some feeds in this baby because I knew it may not be for quite a long time before we can give the baby enteral feeds. So I ended up doing a an androgen ostomy knowing that I'm gonna be dealing with significant fluid losses and so on. But it was the best option at that point and left everything else the same and then came back, uh about four weeks later and it's amazing how things are better. Usually in four weeks, just like gastroschisis babies, they look like they have terrible matted bowel, but you come back in 34 weeks and the bowel is OK to work with. So here we immobilize the jejunostomy and we found just a big clump of dead bowel, uh really nothing to preserve and then reconstituted at the colon. So, and this is a uh an example of how sometimes you really have to think way forward. In other words, I'm not going to be able to solve all the problems today, but I'm going to try to do what I can today and I know I'm coming back. So in this baby, we did a jejunocolic anastomosis again, did not taper the bowel. They really didn't want to do anything at that point and then came back and you can see the distended bowel here. So it was very interesting. The problem with this patient isn't so much that there was fluid losses or any of that. The problem is that patient kept getting infected broviac by translocation. So, there was significant overgrowth in this distended bowel and we kept getting almost every two weeks a a Broviac infection. So we went in, did a step procedure on this patient and the step procedure both lengthen the bowel and plicate the bowel. And that's important to understand that it's really not just a bowel lengthening procedure, but it's a very nice way to deal with very distended uh bowel as well that may not be functioning or that may be having problems of overgrowth. So here you see, and you hear, you see, the anastomosis was widely patent. So sometimes when babies behave like that, people say, oh, it's an anastomotic stenosis very rarely. Is it an anastomotic stenosis? Uh II, don't recall really seeing that uh in a latent way. Usually it's a problem of the proximal loop that is not contracting well. So the step procedure takes care of both. And in fact, within a couple of months of doing that, we were able to get this baby off TPN completely and get the Broviac out. Now here, you have a really challenging case. You have it, it's the type four, but it does have short bowel. So you have multiple atresias, you have about 20 centimeters of ileum before you get to the colon and about 15 centimeters of jejunum and you have multiple atresias. So what do you do here? So there's not one simple solution, but again, using those principles. I wanna try to reconstitute the bowel, but it's clear that I'm not gonna do for anastomosis, one of which is going to be an, you know, like a 6 to 1 discrepancy. This bowel, um, was really not enough, there wasn't enough length to really do much with it at the time. So what we did left the jejunal closed, did the distal anastomosis and put in a tiny feeding tube from a mucous fistula all the way to the cecum to stent. These anastomosis left things as they are, then came back, studied those at four weeks of age and they had all healed fine. So we went back and we took that loop, we stepped it to lengthen it and taper it and we anastomosed it to the mucous fistula and put a gastrostomy. And amazingly that patient had 40 centimeters, about total, increased to about 50 with a step. And by one year of age he was off TPN. So, um it's really, uh you know, you, you, you really have to plan forward for how to uh try to get these patients off. This is a type two ileal atresia. And the principle is as you get more distal, the challenges go become less because usually you don't, it's easy to sacrifice the very distended loops. You don't tend to have short bowels. So many of these challenges really become less. So this is a type two distal atresia. Uh and of course, it's a simple resection, primary anastomosis, same thing. This is a type three a uh ileal atresia with a segmental valve. So you can afford to uh remove the ischemic segment and do a primary end to end anastomosis. And I show you this case because it's one of the most fascinating cases I've had to deal with. So this was a 28 year old man who had had a Jejunal Atresia repaired by a pediatric surgeon uh at birth and was in the children's hospital for two months. It took me several weeks to get his records was in the children's hospital for two months with feeding problem, but eventually fed well and was discharged home and did very well for more than two decades, did really well. Um no issues whatsoever. And then around age 25 started to vomit bile, went through multiple operations, license of adhesions, anastomotic stricture repair by general surgeons. I happened to be giving grand rounds on intestinal atresia. When the general surgeon who had been had just been referred to said, oh, I think you're talking about my patient. And amazingly when we went in, this is his study before you can see the duodenum proximal jejunum. I was able to get the operative report from 28 years back. And um and this patient never had tapering. And when I saw him, he was really in bad shape. He had had a gastrostomy, a peg placed that was constantly draining bile because of his obstruction and he was on full TPN. So here what we found again, severely dilated Jejunum, no stricture whatsoever. So we tapered this over a large foley all the way to align tris and I have a seven year follow up on this patient. Uh, never had problems again. You can see the difference in the study before and after. So this is what I mean by a latent obstruction, you really sometimes have to think way forward. This is another really interesting case. So this is a baby who was diagnosed prenatally and was sent for a prenatal suspicion of Hirschprung's disease. And I, this was the only time that I've ever seen a prenatal consult for Hirschprung's disease because as you know, Hirschprung's disease cannot be, uh, really, uh suspected prenatally. The, uh, perinatologist, the maternal fetal medicine specialist thought it was because while we often teach students that you can't tell the difference between small bowel and large bowel in the first few months of life prenatally, they often see scalloping of bowel like you see here and they know it's colon. So he was suspicious and this was the only uh, distended segment, a colonic segment. So that's ok. Let's see what happens. So, at age six hours baby with scaphoid belly, uh really no significant problem except a bit of a paucity of gas on one side. But bowel is not distended, but lo and behold 12 hours later, baby is distended and we have this immediate contrast, enema does not reflux into the small bowel. So, blind ending colon, and we find this problem here. So this is a colonic atresia in the east ending colon. And it, it's really interesting. I've had several of these patients now and in at least a good number of them, the ileus valve, I'm not sure why it tends to be competent. So these patients can actually have a closed loop obstruction. So again, people talk about, you know, doing an, a temporary colostomy, tapering the colon. I don't really see any reason for this. You're not trying to preserve colonic length. So here just did a uh right hemicolectomy or right colectomy basically removed the atresia and then did a primary anastomosis of uh ilium tool. Now, in this case, in colonic atresias, if you look at all the textbooks, they talk about Hirschprung's disease, you have to think could this be Hirschprung's disease? And I think that's important, but it's overstated because the incidence of Hirschprung's disease in colonic atresia is around 2 to 5%. It's not high, higher than that. Nevertheless, before you anastomose, you should send part of the distal margin, the colonic margin for ganglion cells. And I think if you're suspicious and you don't have that ability to get frozen, it's probably safer to do a temporary stoma, wait for your finals and, and come back, here's another patient we had recently and now in this case, there's an incompetent valve very similar to the first case, except in this case, the obstruction is back feeding into the distal ileum and the surgeon. Uh it wasn't, this wasn't my case and this is the colonic reconstitution. The surgeon in the decided to do a uh bishop who anastomosis and that worked very well. II am not super fond of this anastomosis. Uh because I do think that you can still plicate taper the bowel and do an anastomosis for most of these patients. But this is an option when you have significant uh size discrepancy. So the summary of what I wanted to say is that you, it really helps to think of those three guiding principles. You should have a methodical operative strategy when you go into these patients and sometimes you need to do a tailored approach, meaning like I'm going to really customize it to this patient. And I'm going to, I think to what I'm going to do today tomorrow and potentially beyond often patients is rewarded and the outcomes really for these patients in an environment where you have adequate support should be excellent. I do wanna make one last point about intestinal atresias. Uh We're actually trying to lobby and call this the Gutman syndrome. After one of our predecessors here who passed away recently, Doctor Gutman described a syndrome of multiple intestinal atresias from the pylori to the colon and those patients uh are familial. Um They have been reported here in Quebec, but there have been multiple reports from Europe. I cannot recall if this has ever been reported in Africa. But what you see in these patients is calcifications throughout the abdomen and a gasless abdomen. And really, if you know about this syndrome, those patients should be palliated. They have no bowel to preserve, they usually have 50 60 70 atresia throughout the bowel. And we, this really should be called the Gutman syndrome. So just to the trainees, um, I'll give you the same advice. I give my trainees, your purpose and training should be the competence to treat the disease primarily, but it should not end there. It should also be the complications of the disease and the complications of the treatment of the disease. And these all are, uh, you know, fall in different categories also on pediatric surgery. We deal with a lot of very rare phenomenon and, and you do not have to worry to see everything even in the center. I'm sure it's yours. That's very busy. You may not see everything but one of the beauties of our specialty. Certainly, why I entered it is it's a small community. We interact with each other and we still help each other with cases all the time. And finally, uh I just want to put a little plug for this CB clips. It's on Twitter, also on linkedin and, and Facebook. I hope as many of you join and interact as possible. It's been a wonderful way to share and learn together. And if you want to do a case, if you want to present a case, this is really my purpose for this year is to have others guests present. And it's very, very simple. You just tape. Uh I mean, I I'll help you through the whole thing. You just essentially do use your iphone to record a two minute video, uh a short powerpoint and I'd be very happy to host you as one of the uh host of the CB clips. So, thank you so much and I hope we still have time for some interactions. This is how our city looks today. Uh Last night, I'm sure you're gonna feel very, very fortunate where you are. But uh we will get to a summer where uh it looks like this. So I hope to visit you with you sometime and I certainly hope to see some of you here. Ok. Thank you very much, Sheriff. That, that was uh quite interesting, informative and educational. Uh We uh as you seen and as you, uh as you see, uh Professor Larry Headley is with us and Professor Colin Lazarus who was our uh ex head of the department and our mentor is also here. So, uh I will invite Professor Hiley to give his thoughts. Um and, and his comments, please. Yeah, yeah, sure. It's good to hear you again. Um And uh thanks, thanks for a very thought provoking, uh, presentation. Um, you, you're bent for history. It, it was worth noting that at the time of that report from, uh, low that, uh, Chris Barnard was a pediatric surgical registrar. Um, so he was actually planning a career in pediatric, didn't, didn't make the grade and, um, went on to, uh, to train as a cardiothoracic surgeon. So, for all the trainees, uh, don't worry if you don't make it, I think. Right. Larry. Well, I think he did the first heart transplant. He did indeed. Yes. Yes. Um, that, that's, that's where people who don't make the grade in pediatric surgery. That's where they end up. Um, als also, I think, um, we owe a great debt to, uh, Hh Nixon, Harold Homewood Nixon who, um, was really the guy who pointed out the problems with that dilated bowel and there is no doubt that if it's surgery, you flick the bowel it'll contract. Um, but it's, it's a nonpropulsive contraction. And he showed for a very elegant series of operations on, on, on, of experiments on isolated bowel that this, um, dilated bowel was an ineffective propellant. And so the whole thesis of resecting boldly resecting the proximal bowel came into being. And then since then, of course, we've had the tapering and the delore the uh inversions, et cetera. But, uh, we, we, we work in Africa. Um, and I would, I'd like to know what your approach is to the patient who pitches up. He's um, he's 23 days old. He's got Gal Atresia. He looks like a sticker built on. Um, he is, shall we say a poor operative risk? Um And TPN may not be available. Um And in fact, even for treating your, your, your, your straightforward um uh internal atri TPN is probably not going to be available for many of the people on this uh participant list. So, you know, we, we've, we've fussed over should we give these people preoperative TPM and try and get them fit for surgery? And as you say, they all get uh broviac infections and shuffle off this mortal coil with uh septicemia. Should we operate on them? Um And then try and give them postoperative GPN or try and get them early feeding trickle feeding to try and tide them over and uh they starve to death. Um And so, whilst in the developed world, um small Basia might indeed be a done deal um in Africa. And I suspect in many other parts of the developing world it's still a major problem. And I think we've got to reconsider our operative approach. And I think there may well be a place for uh feeding stoma and um a, a dis, you know, putting in a distal feeding catheter and a proximal genital atresia, um, et cetera and, and the bishop coop. Um, you know, because you can, you can feed the baby down the distal limb aspirate, what comes up the nasal gastric tube and stick it down the distal limb and it's, it's going to be sort of what you would regard as rudimentary techniques like this that are gonna improve the very significant mortality um uh in Africa. So, how would you approach it? Yeah, I completely agree. Larry. In fact, if you look at the history of these types of anastomosis, the cent the Bishop coop, I mean, these were uh used essentially when we still had, we were in those settings, like we didn't have really that type of new needle support, uh uh TPN support, et cetera. And I do agree that uh often because you have venting chimneys and so on that they do lead to earlier um earlier intervention now and I've operated in Rwanda and other places with surgeons who face these dilemmas. Um For example, let me, let me just give you AAA correct example, type one. Usual Atresia. I mean, first of all, I'm uh uh usually amazed that these babies have survived a couple of weeks without really any nutrition, we can not able to absorb anything and I've avoided the resection altogether, you know, basically do uh uh what, what I described for do the Atresia and I often in very, very small babies would use a, a smallest catheter possible and feed distally as soon as possible and that's their own. And, and despite that, the mortality is still extremely high and I think the question really becomes an ethical one if you have a very late presenting baby who, you know, is already in severe malnutrition, maybe even septic and so on. Is there really a role for surgery at all or is really palliation the right way to go and not subject that baby to any intervention? And I, and I don't have a, a right or wrong answer to that, but it's definitely worth at least considering. But I do agree with you that many, much of what I talked about are really meant to be for a by resource environment where the TPM support is available if needed, where you know, you have good NICU support and so on. And, and South Africa is probably not an example but, but for really the the lowest income countries and where resources are very, very tight. Um Your focus should be uh uh how am I gonna get this baby feeding very early? And I do agree that at least in my uh e experience with this that something like a bishop coop does tend to, to allow you to feed uh sooner. So certainly it may be, be very well an option for many of these babies. Yeah, thanks. And with regards to Duodene Latris, I mean, we looked at our patients are several years ago now, but in our environment, uh interstim malrotation with the midgut volvulus is far more common than Duodene Lasia. And so if you're gonna make a mistake in this neck of the woods, then you should make the mistake of operating emergently and finding that. In fact, it's a Duodene Lasia. Um, and, er, er, just to, er, remind our speaker that, that malrotation without a volvulus doesn't present. Um, you know, it's, it's asymptomatic. So you're never gonna, that if you've got a patient with malrotation who's symptomatic, he's got a Volvulus, he's in danger of dying stat him. So, uh really, you, you've, you've got to get on and, and do it. And the other, the other thing that interested me was th this concept of a subumbilical incision. I, I've never done one well, II mean, I've done a fan and steel but, um, II don't see the advantage of a subumbilical transverse incision getting a, um, a transversal approach to the, to the bowel. And it's, it's interesting that you should present that uh uh paper by Richie Ricketts. Um II was in Atlanta and uh watching Richie Rickets, um, trying to learn how to do a laparoscopic fundation and, um, uh he, it was Vulcan who was Vulcan who was teaching it. And uh like me, he gave up, he said, I'm too old for this. My arms are aching. I can't say I can't judge distances. So he went back to doing them open and I think that, um, uh just because something can be done laparoscopically doesn't mean it should be. So I, that's a II think that's a great point and it's important for the trainees to really look at all this. Uh what, what we think is dogma to look at it critically. Uh, you know, um, so we don't do a lot of fundoplications in Canada. We're very conservative. We don't, you know, the operation is done very rarely, but I have stopped doing uh lab fundoplication in small babies in the NICU, you know, the 34 chemo babies. I stopped because I do think the open operation is gentler and here's what's really amazing. So we present the laparoscopic fundo as you know, the state of the art. In fact, the whole, it i the whole re um area of gastroesophage reflux has very poor literature and surgery, but some of the best literatures actually open versus laparoscopic fundal. There are about four or five randomized controlled trials and I summarize them in my chapter and Reflux and guess what? There has never been an advantage to laparoscopic fundoplication actually demonstrated in any of these. So again, you have to look at things, um, it a bit critically and not be accepting of, um, just sort of, you know, what becomes, uh, what becomes surgical dogma. I couldn't agree more. Thanks me. Yes, thank you. Uh, now I just invite, uh, Professor Colin Lazarus, who is also one of very experienced surgeons and, uh, I know he's quite fond of transanastomotic uh tubes. Um, even in cases of uh small bowel aeria and, and please call in, you can make your comments. Yeah. Yeah. Ok. Call in. Yeah, colleagues involved in dog training. By the sound of it. I don't know whether he's able to hear me or not. I can give one more try. Hello, Colin. And uh there seems to be some problem. I think he's probably unable to hear me. Uh Next I will uh invite doctor Itai Sang, who is a consultant, pediatric surgeon and uh in charge of the unit in Port Elizabeth. But he has a very strong association with our department in East London. Uh more than 20 years back and he uh uh trained as a fellow in periodic surgery and worked as a consultant here and only about five years ago, he relocated to four days a bit. So it uh your comments, please. Uh Thank you. Can you hear me? Yes. Yes, we can hear you. Um We have had one patient with a quite approximal vaginal atresia um about 25 centimeters be beyond the ligament of present. Who is interesting was interest in that. In that the baby presented it between 4 to 6 weeks of age and like the sometimes the baby, the baby, there's a lot of maybe just maybe just Hello. Hello. Go ahead, go ahead. Uh Can you hear me? Can you hear me? Yes, we can hear, you know, if that could be muted. Yeah, we could try that. Uh uh How is it now? Good. It's good. It's good. OK. I was saying we had a late presenter as well of a vaginal atresia with probably between 2025 centimeters as the level of the atresia beyond the ligament or right. And the child presented it almost six weeks and one wonders how the baby survived because even the um the blood sugars were reasonable. The baby was not hypoglycemic. So there must be some absorption that went on in that proximal gut. We didn't try to do anything fancy. We do have uh TPN in Port Elizabeth is everyone knows probably o other than uh Professor Emil. So because we didn't try to do anything fancy other than the initial day or two of resuscitation and getting urea and electrolytes corrected and so on, we repaired it because we felt we had no other choice the usual way as if the baby had presented early and decided the baby would take its chances. So we did a repair, put a central line and once the nasogastric aspirates were low, we started slowly feeding the baby and the baby is at home. I can't tell you why the baby survived. We certainly don't have a serious, it was just one lucky patient we had, but the baby survived and is at home. The interesting thing being, of course, the baby presented at six weeks old and had survived. You know, I won't go into a long story. It's a long story how the mother thought why it was, felt the baby had nothing wrong for more than a month but the baby survived in all, all done. Ok. Yes. Uh, thank you. I, um, uh, again, um, I think, uh, Sheriff, uh, our problem here is, uh, is, uh, we can give TPN but after about 68 weeks of TPN, we run out of lines and we start getting complications of cholestasis, uh, sepsis. And so we personally haven't done any step procedure. Um, I would like to ask, uh, prefer if he has done any step procedure or he knows of step procedures being done in Durban, prefer. Yeah. The Hymen. Yeah, I know, said procedures being done in Durban, II haven't done one. I, not many of my patients live long enough to have one done. Um, but, uh, Sam Shak has done um, a number of bowel lengthening procedures, um, with, er, relative success. I'm not sure that he's actually managed to get anybody off TPN in his practice. He has a, a couple of kids on home TPN at the moment. But, uh, certainly the, um, uh, interstital absorption has been improved by the step procedure even if it hasn't completely solved the problem. So maybe I can just make a couple of uh, comments about the step procedure. Um, you know, the step procedure was invented by a medical student who's now a pediatric surgeon in Bos Boston. They, Kim and it, it, one of the reasons. Uh, you know, when I read about it, I really thought it was, uh, fascinating is, you know, in the past when we used to do the bianchi, it was always nerve wracking cause you know, you're dealing with short bowel and you're trying to go through the Mesenteric pet um sheath and, and really worried about potentially damaging more bowel. And the nice thing about the step procedure is that the chances of causing bowel damage are really, really low and it's really pretty, pretty easy to perform. Um, it does not disrupt the mesentery in any way. So there is really no, uh, significant feasibility of getting bowel damage. However, the question is when, and I think this is the point again, Larry's point is excellent. Like, how long do you need the patient to survive? So, if you are going to do a step procedure, because a lot of people are talking about primary steps, you know, at the time of exploration lengthening the bowel right away or early on. The problem is if you take bowel, that's, let's say, total of 30 centimeters, you know, let's say you have a 15 centimeter jejunum and you make that, you know, 25. Well, you're still gonna have short bowel, you're, you're not gonna get off that patient off TPN. So where it really helps is in those late patients who are trying to get off TPN, you know, they're not far away and giving them just an extra competent of will, will tip them over. That's where I really feel it becomes helpful. And the point is you can't really get there right away. You have to usually wait at least until the patient is nearing a year of age or more. And there in lies the, the problem of still having to support them until they get to that point. Yes. Uh Thank you. Yeah. Uh Now I will invite Doctor Majola. Doctor Majola is uh one of the consultant pediatric surgeons and he has done recently a case of uh type four gingival atresia. Um uh the baby is recovering in the PICU Doctor Majola. Thank you. All right. Thank you for, for the invitation and thanks Christal for a good um presentation and also to prof uh amil for his wonderful and insight and also informative uh presentation. Um Thus far, I have no comments. Thank you. Ok. Thank you. Doctor Doctor is uh uh our consultant pediatric surgeon who trained uh with Professor Adley and has been with us for about 3.5 years. So if she has any comment, uh yeah, sure that hi Prof. Yes, thank you. Um And thanks to Proil, that was really good. Um and lovely pictures to see and thanks for telling us how they do it on, on their side of the pond. Um I would just like to si and from Prof Hadley's comments, you can clearly see who trained me because I still believe in the bishop co as well. So, and I have do have done it on a few patients here. Um I just wanted to ask if any other consultants from other African institutions are on what, what they prefer to do if they don't have any TPN and they need to feed the patient early. I see doctor Taurai Zuno from Zimbabwe, Taurai. Um any comments. Uh Thank you prof um Well, uh as you already know, you know, South Africa is uh Zimbabwe supermarket now and if South Africa doesn't have TPN, you can, you know, it goes without saying what uh what's happening up here. So we are almost always out of TPN. Uh the few times that we do get it coming from South Africa and it will be awfully expensive for most of our patients. So it's really quite a difficult situation for us. And uh unfortunately, we needle needlessly lose some of our patients because we, we don't have the, the appropriate interventions. Thank you. Thank you. Do. Right. I see. Doctor Fun Kolo is also here. Doctor Kolo is a consultant, pediatric surgeon in the third unit in the Eastern Cape province. Uh He is in charge of the unit in Umtata uh Fun. Uh Do you want to share any experience, make any comment? All right. Thank you very much prof and uh thank you to the presenters and the, and the visitor prof uh from our side uh we do have uh access to TPN so that take a little bit longer to, to arrive at in the audit from Port Elizabeth. So, um I think much of the problem that we experience here. Uh It's similar to what doctor if I have said that uh most common, it's late presenters, but once we resuscitate them and we operate them, the the outcomes are fairly straightforward to previous, the outcomes are fairly, are fairly good. I would say other than that, um Thank you very much, Doctor. Thank you for the platform. Ok. No, that's, that's perfect. I think I'll just uh request uh doctor Emil to give his final thoughts and then we can close the meeting, Sheriff. It was really great having you. We are certainly going to rope you in for some future meetings. And please, if you think they are appropriate for your friends in Africa, uh please feel, feel free and, and share uh the Google uh form to register for the meetings. Our aim is to spread education and uh establish contacts with our colleagues in Africa. Well, uh I just, I really wanna uh applaud your efforts and then, you know, uh despite the doom and gloom of COVID and all the things that we've gone through around the world, I think one of the things that's taught us that we are a smaller community than we believe we are. And I know that a lot of these meetings uh you know, weren't happening before because it was a big deal to travel and, you know, I mean, it was very nice still to see each other in person. I don't think that's ever gonna go away but I think, uh, COVID has made us a much smaller, um, community. Uh, and, and it's just wonderful to be able to have these types of participations to really benefit all of those who, you know, uh, don't have enough time. And also, um you know, don't, don't have the, the means to always uh travel to large meetings and so on. So I'm very honored to be with you today and I look forward to future uh meetings by Zoom and, and hopefully one day uh see you all in person. Thank you so much. Thank you very much, Sheriff. Uh Thank you all. Thank you Crystal for nice presentation eai for mentoring uh pro and pro and all our consultants and the delegates who are attending next week. Uh We will have a talk on COVID and PEDIATRIC surgeon. So it's quite going to be quite an interesting talk by one of our uh medical officers and we'll have a pediatric infectious disease specialist as, as an invited guest for that talk. So, thank you. Have a good day. Uh And bye-bye, thank you, bye-bye.