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Ga do you just want to try sharing your screen? Yeah, that's good. Um, it's good. Yeah, you can stop sharing just for my intro and then, then start sharing again. Hello, Ellen. Good afternoon. Hello, Ellen. Good. Hi, Hon. Yes. Good afternoon. Uh, hi, how are you? I'm good. Good. And you? I'm fine. Thank you. We'll just wait a couple of minutes and then we'll start. No problem. Ok, a week and. Mhm. John. Yeah. Ok. Yeah. Ok. Mhm. Ok. Good afternoon, everybody. Um, welcome to uh, today's meeting um, of our department of pediatric surgery. The topic is going to be gastrointestinal duplications and we are glad that that doctor Ellen Mauna has agreed to be invited guest for today's meeting. Uh, Doctor Mauna, uh did her undergraduate studies uh at various places, prestigious places including Saint Andrews University in Scotland, uh University College, London Medical School and also in Malawi. She did her pediatric surgery training uh between University of Pretoria and Wits University and she is now currently head of the pediatric surgery department at Charlotte that is previously known as Surg General Hospital. Uh, these are her words, which I really liked. She says that I love teaching because I learned from it. My patients have been my number one teachers followed by students at all levels. Undergraduates are my favorites and of all achievements and accolades. One I'm most proud of and grateful for is Mama Themba. So welcome Ellen and welcome to all the guests. Um and and um attendees, we will start as soon as doctor uh hil in the house or Registerr will be able to share a screen Elga. You can go. Mhm Hello everyone. Um I can see the slides properly. You can see the slides but your voice is a bit, a bit soft. OK. Let me try to make it better. Let's see if this is better. It is better. Yeah. OK, good. Um I'm he now my topic for today is gi tract duplications. Why are they not going for it? Um Slide full screen Elga, sorry, just make your slide a full screen slide. Let me try again. Yeah, that's good. Good. Um Just some background. It's uh G IDG I tract duplications are actually quite a rare condition that we don't see very often. They come with a very varied presentation depending on the location and the type. And as we'll go through this presentation, I'll show you where all they come and how all they present and generally they comprise of a well-developed smooth muscle coat with epithelial lining. Um that and a mucosal lining that represents the adjacent um gi tract and is generally attached to the um gi tract where it's coming from. Um just some embryology. Unfortunately, there's no one theory that explains all ge tract duplications. Um Therefore, the current assumption is that there is a multifactorial process um that is associated with some vertebral spinal cord and genital urinary and ectal malformations and possibly even with intestinal atresia. Currently, we have five theories that explain some of the gi tract implications that we see. The first one is partial twinning. Um This is where organs or multiple organ systems can be duplicated. It's often seen with hindgut duplications who are associated with genitourinary duplications. Um So two vaginas or two vagina, um sis with a rectal um duplication of a rectal cyst and a split notochord theory. S um says, well, it starts off in the first month, the notochord separates and the theory says that gaps in the notochord develop that let endoderm herniate through and form diverticula. These are especially um pre or seems to be more common than the ones with spinal defects. So, higher duplications in the thoracoabdominal region or only thorax region. Um and these duplications can connect to the spinal cord and need to be kept in mind when we try and manage them. The third one is diverticular defects. Um These are thought to be persistent, embryonic diverticuli that form from the gi tract. This was the first formula uh first theory that was formulated. Um however, it's mostly seems to be rejected. Now, as diverticular form from on all sides and surfaces of the bowel and duplication, cyst generally form on the mesenteric side of the bowel. And diverticula don't often contain the same amount of wall and epithelial tissue as the duplications that we see. The fourth theory is the second one that was postulated. It states that the organs start as solid tubes and in the process of forming movements to evacuate during this process, diver particularly form and they usually regress during fetal life, but some might persist until later on. I know the form duplications. And then lastly because the first four don't quite explain all duplications that we have see. Um seems to be any hypoxic vascular incidents or trauma that happened during the embryological or fetal stages. Gi uh geo duplications are often found incidentally, we do something else and happen to see that they're there. They can be diagnosed antenatally and in the first world, they commonly get diagnosed antenatally in our setting. Unfortunately, we often only see them once they present with symptoms, they can present with a range of symptoms that start from pain, obstruction, um abdominal distension if there's any obstruction that may present as intususceptions, bleeding or perforation. If there's ectopic tissue present or respiratory compromise with recurrent pneumonias or shifted mediastinum. If it's of thoracic duplication, the present presentation mostly depends on the size and the location and whether or not heterotopic mucosa is present in our settings, they usually present before two years of age. However, sometimes they only present in adulthood with complications of the duplication cyst, they are generally classified into for gut, mid gut and hindgut. Um, duplications start anywhere from the mark down to the anus. They, the most common ones that we see is the gal E ones which present about half of them that we see. Um, next one would be the esophageal ones. And then the third most common is the colonic ones. They can present either spherical or tubular as you can see on the picture. Um, here's my arrow here, some spherical ones and then tubular ones. Um, the stomach one is not quite correct because it's often a spherical one and not a tubular one, but it's generally on the greater curvature. We then find some thoracoabdominal ones, um, that are not classified together with esophageal ones that often track from the esophagus down to the, um, forgot structure, the stomach duodenum or sometimes jejunum and may or may not be connected to the spinal cord and then some can contain ectopic tissues. Um, that is usually pancreatic or gastric tissue. These can be an esophageal duplications, stomach or duodenal or gal and ileal. Usually colon and rectal don't have ectopic tissue and then do some investigations. Investigations are quite difficult because of the varied presentation. Generally, it's recommended to start with an ultrasound. It's noninvasive and it's relatively easily available. Um, it's also used for antenatal diagnosis, if we do find them antenatally on ultrasound, um we can see this double wall sign um as these cysts have the muco of the muscular layer and therefore thicker walls than normal cystic structures that we see in the abdomen, like mesenteric cysts or mental cysts. Um And all over the world is the first investigation that we do. However, x-rays often get done first just purely on the no the nonspecific presentation that these Children can come with. Um And if ultrasound doesn't show anything, x rays are our next go to anyway. And they're generally not very helpful, especially for abdominal duplications. However, in the thorax, you might see um uh structure in the mediastinum or something that's pushing the mediastinum to one side or the other, um or can show pneumonia and complications from these duplications. The next recommended investigation is contrast studies. Um They may not show spherical duplications but they can show tubular duplications. Um For clerical ones, there may show uh filling defects and they're especially important in rectal duplications as there might be a fistula to other organs or to the skin from the rectum. And these need to be delineated before we start operating on them. However, these first two studies don't often tell us what exactly is going on and therefore the next recommended one, especially in the first world is act scan and our African go to one if we have it available and some of the other African countries. CT scan is not readily available and they will then skip the next few investigations. CT scan can be very useful as you can see where the structures um are located. Um plan operations, see what other um associated structures are close by. On CT scan, we um can confuse these um duplications with other ring enhancing lesions due to the muscular layer. They take up more contrast and therefore, um look similar to ring enhancement le lesions. These need to be differentiated from septic reasons um with a septic workup, ABC and ACR P. And if the child is nonseptic, um there might just be a duplication, cyst and not a septic origin. However, in our setting, that can be very difficult as our patients present immunocompromised and often with TB. Um but then we'll have to investigate and see what it is. Um MRI is often done in the first world countries, not so often in our setting. However, if there is a duplication cyst involving possibly involving the spine, MRI is very useful to see if there is a connection. And then lastly, the technetium skin gray is recommended to check for ectopic mucosa as that's important for planning operations, especially if we have duodenal duplications, then coming to treatment um just some principles and then I'll go through some more detail. Um And ideally, we would like to excise the duplication completely. Um However, the surgery should not be more radical than the problem for the child. Um Therefore, for especially duodenal uh duplications where operations can be quite complex, we will have to find the easiest solution with the least morbidity for that child. Keep in mind that these duplications are often on the mesenteric side and share blood supply with a native bowel and that can complicate surgeries. Um Remember that there might be he heterotopic mucosa and that ideally need to be excised. Um depending on where it is, there are some other options available. And then keep in mind the relationship of adjacent structures, which is especially true for duodenal duplications with ability, tree and pancreas and for the rectum, especially if it's anterior duplications as that will make our surgery very difficult. And we have to think of different options than to just excise the duplication for more specific management. I'll just run through the different areas. Um, as I said earlier, they can present anywhere from the mouth to the anus. The oropharyngeal ones often present with a mass. They usually a cystic lesion, not a tubular one. And the recommended treatment is to drain them into the oral cavity. And that usually solves the problem and cervical ones can present a cystic masses. Um There is a very long differential as to um what the cystic mass in the neck can be and usually needs quite a bit more workup than the oropharyngeal ones and they usually excise through the neck. Um, completely esophageal ones are the majority, they generally are located on the right of the chest. Um True, true esophageal are usually cystic. However, they share the muscular wall of the esophagus, which can make excision a bit more complex. Um They need to be removed as they often become symptomatic and can or even form malignant tissue as they can have ectopic heterotopic tissue. And they can be approached either through a thoracotomy or if the skill is available. Um with thoracoscopy, if the esophageal ones um have spinal involvement, generally, the neurosurgeons need to be involved and they start off with the procedure to ligate the connection to the spinal cord and then we take out the rest of the cyst versus to prevent infection going and causing meningitis into the uh spinal cord. Um Thoracoabdominal ones are luckily re relatively rare. They often also sit on the right side but they can communicate into the abdomen and to the intestinal tract distally, they're usually tubular and they have a very high percentage of gastric mucosa, which needs to be kept in mind when we do these surgeries, they are usually done with a two stage approach um where we start off and remove the thoracal um thoracic part first and then the abdominal one. However, it needs to be made sure that this drained somewhere as otherwise it can cause infections and keep in mind that there is often heterotropic mucosa, which also needs to be stripped to prevent complications. Um gastric duplications, um are usually symptomatic early. They can present um with a similar picture to um pyloric stenosis. Um, but they don't, can be at the pylorus but often on the greater curvature, they're usually cystic and resection is recommended and it's usually relatively easy. Duodenal ones are likely relatively rare but are quite complex. They can have varied presentation with from ranging from gastric outlet obstruction to jaundice to recurrent cholecystitis and pancreatitis. They also have a higher percentage of ectopic mucosa that can cause ulcers and perforation and can be quite difficult to excise. Um Given all the structures that are so close to the duodenum for this, we need the most experienced surgeon to do the operation and keep in mind that there's multiple options. Um One of the one more recommended is to try and um excise the mucosa and leave the rest to drain into the lumen as it doesn't come with as much morbidity. But that depends on where the duplication is situated. Small bowel. As I said earlier is the most common duplication. We see it can either be cystic or tubular. It also commonly contains heterotopic mucosa and resection is the most recommended treatment. Um That's usually done with a localized resection of the bowel and in primary anastomosis as they often the duplications share the blood, sorry, share the blood supply with the native bowel. They can be mistaken for Meckel's diverticulum. However, Meckel's diverticulum is situated on the anti Mesenteric side and the duplications are situated on the Mesenteric side. If we find one small bowel duplication, we must just check the rest of the bowel for any other duplications. Um, and excise them. Also, keeping in mind if we have to excise a lot of small bowel to get rid of the duplications, we, um, might excise so much that we have a short bowel. In which case, we have to come up with a different plan. There are also a few options available for that one. For colonic duplications. Again, they can be cystic or tubular. Um, tubular seems to be the more common one. The smaller ones again are resected. Um, locally long tubular duplications form a challenge. Um Luckily, they don't usually contain heterotopic tissue and if they can't be excised, it's just recommended to drain them distally so that they can drain it, don't form obstruction. And then keep in mind that there might be fistulous tracts either to the skin or adjacent organs that need to be excised as well. And then rectal ones are also quite a challenge. But luckily, um, not that common and amongst the rectal ones, the most common one is a presacral. Um, and that's situated on the posterior rectum. Keep in mind that these ones can present with genital urinary and, and ectal malformations. And the surgical approach depends on the location, generally recommended as posterior sagittal if it's localized to the lower rectum. Um, sometimes a transanal approach can be used otherwise, like in this picture where it's quite a complex and long a thing that's connected to multiple things might need to be laparotomy and perennial approach. The complex ones are the one anterior recal ones where you also need very skilled surgeon to deal with those because there's so many adjacent structures and it surgery in that space can um damage vascular and nerve structures going down to the pelvis. So, in conclusion, um duplications present in various ways, likely they're relatively rare. They come or can occur from anywhere from the neck down to the anus and can be simple cystic complex, multiple tubular, they can be associated with other anomalies, including the spinal cord anomalies and genitourinary anomalies. Um optimal treatment or recommended treatment is resection and they generally have excellent long term outcomes and quality of life. Thank you. Are there any questions ga excellent presentation and uh and a very nice overview of, of a rare but complex uh topic so well done. I'll now invite uh doctor map to, to make her comments, Ellen. Do you have a powerpoint or you will just stop? Um Let me see if I'm able to share. I enjoyed the presentation. Yes. Um a good summary. It's true. It's very rare. I mean, surprisingly, they caught it at one in 4500 which I think that's not rare enough. We had received these things as, as Helga was uh talking. I try to recall other cases that I have seen, like, I've only seen one in the mouth that we actually thought it was a, a demo cyst. But the pathologist told us no, it was actually a duplication cyst uh from the mouth. But yeah, the, the Jejunal area was seen. And I also so recall like one case of uh a bigger child, adolescent of 12 years who presented with an acute abdomen with free air and she actually had a perforated ulcer, but with a duodenal duplication right next to where she had the perforation. I'm just going to try and share when I saw the topic. I thought um I put, what do I do? Chest pain. Mhm. Thank you. I, if you just go right at the bottom share screen, yes. Um I just wanna open the, you know, presentation. Yes. Yeah. The other comment I had is that um they, they don't wish you already stayed. They don't have any um way of, of uh presenting. It can be, is my screen showing. Uh Not yet. OK. So like the, the presentation will depend on the, on the site and you don't, most of the time you won't even have uh you won't have the diagnosis until you go to theater. OK. Yeah. Am I still on? Uh Yeah, you, you, we can hear you talking but uh we can't see your screen yet. OK. This is bad. So if that presentation is open, you can just go to share screen. Yeah, that's what I, well, I've got my presentation on, on the thing and then the Zoom is gone at the bottom. There is a green arrow. You just have to click on the arrow to share screen. I'm sorry. No, we just at least go back to Zoom and this is the tri. OK. Yeah. So I've got my share screen. That's OK. There we go. Is it on? Yeah, it's perfect now. Yeah. OK. Now, so just, just for yeah, the purpose of yeah, teaching and sharing. I wanted to give you quickly two cases. Just recent cases on duplication since that. Yeah, we have seen this side. So this was AAA four days old uh post big baby that the pediatrician actually kept that long because she thought uh the baby is gonna pass too. But this baby hadn't passed stool and the abdomen was starting to distend. She sent me these x-rays like uh as you can see, there is some distension of valve. This is suggestive of uh distal intestinal um obstruction on the lateral. You can see maybe uh if you see my point, uh you can see some suggestion of stool there and again, dis to intestine obstruction. So now when the baby arrived from another hospital, that was what I we were thinking, OK. It's a dis intestine obstruction. And then they said the baby was not feeding. Well, my thinking was, you know, this baby has got a distended abdomen. It won't be interested in feeding. And then, uh when I did a di digital rectal examination, there was just some mucus there. Uh And the, the pediatrician had tried also to put a feeding tube she said in and it was stopping. I didn't try that again, but there was a, an open anorectum, but you couldn't go far with your finger. So at this 0.1 is thinking the usual things that we know as causes of this to intestine obstruction. But there's no, there's no uh anorectal malformation. So you ruled that out. But you consider the uh stenosis. This is now, I just asked for contrast, you know, this, it was always the child needed surgery. But I asked for a contrast study. This was the control x-ray. As you can see, you see this quite distended. But, you know, you can make out that it might be something that's a blind ending there just below the sacral uh promontory there that's funneling down but quite distended and uh blind ending. So you think, is this an atresia what's going on? Then we did a contrast study together with the radiologist and they were putting contrast from the last review, you can see the contrast is kind of bypassing that uh blind ending tube. You know, at that point, we are wondering what's going on. I'm just showing you the, the X rays, uh you know, inverting the images and on the AP you can see the contrast is going beyond what you thought is an Atresia. There's still contrast going on in this particular patient. And even a later view of it, you know, almost like you've got AAA sickle, that sickle shaped uh contrast going in. So we wondered and we had long discussions and the radiologist put this, this as his differential diagnosis uh in theater. This is what I found or that we, what we found there was an Atresia. You can see this is blind ending. You're looking down into this, this uh uh diva retractor is at the bottom. You're looking at the rectum down there and this blind ending thing. But when we opened it, it was opened vertically to say, can we open and repair this? Then you say uh uh but something else is going on here. There is this lumen, the proximal colon, let's call it sigmoid. And then you see the rectum, the, the, the lumen of the rectum with this mosquito here, that's the one proximal distal of the rectum. But there's another lumen in between that is not cannulated in this picture. So then one looks at the whole picture, sorry, I think I skipped. Yeah. So one looks at the rest of the anatomy. You can see that there is a duplication, what you would call a tuber tubular uh duplication of the colon. It skips on that bit and then it carries on. Actually the rest of the colon had uh that duplication. Like she already said, it is usually on the mesenteric side. So, what we did is gi step. I liked it when she said, whatever you do should not be, you know, more than the patient needs. You know, we, you don't want to take out all the colon. You can't take out that duplication uh from the mesenteric side easily without compromising uh blood supply. So in this particular patient, we staled with this uh 45 centimeter twice, just to make these two lumens communicate well. And then proximal also just made an area on the proximal transverse colon to make it uh communicate. And that's all that was done. And then of course, the primary anastomosis uh in view of the uh atresia. So this was double pathology, atresia with um um duplication uh of the colon. And then the, the last case just quickly, this child when uh she presented, she's almost two years old, she presented as a prolapse on our meetings. Everything everyone was like there's a child with a prolapse. This thing was mom noticed recently this thing coming in and out, I mean, coming out and back in and uh eventually uh on uh ultrasound and CT scan, they diagnosed uh a duplication. I think this doesn't project well, but it was duplication of the sigmoid as well as rectum. So, what we did is to just, I mean, we examined and maybe we need to do something further. This is very recent. Uh Three weeks ago, we found that there was a fistula into the duplication. So it would fill up with feces mucus, then it will protrude out, then it would decompress itself. So basically, we used that fistula to make it to make the duplication uh of the rectum com communicate with the uh the main rectum and we found we stimulated and checked it was within the sphincter complex. So that's what I wanted to share as to what we we've experienced on this side in terms of uh duplication. Both of these cases are the colon duplication. Thank you. Uh um Thank you, Ellen. Uh very excellent cases and, and uh a very nice practical demonstration of, of diagnosis and of management. So I'll uh a any other comments Ellen about the topic in general? Uh No, I don't have any other comments. OK. Thank you. I see. Uh We have pro hardly uh present with us. Pro hardly any comments from your side. Any different. Yeah. Yeah. Hi. Hi, good afternoon, everybody. Um Yeah, I just like to congratulate uh Ellen for uh spending her part of her undergraduate training at such an excellent university. The University of Saint Andrews. Yeah, I II imagine I was there a few years before Alan but um yeah, Saint Andrews never changes. Um Yeah, II mean, I think the, the cases Alan's presented, exemplify all the problems and I have um I'm embarrassed to say, actually put a gastrostomy into a gastric duplication and, and wondered why it didn't work. But the, the, the commonest ones that we've seen have been in the tongue, um, and you know, the, the cystic duplication in the tongue, it is related to the Foramen caecum, but they are to all intents and purposes, cystic duplications. Um, and those we were, um, assaulted by splitting the tongue in two down the midline and you can easily access the cyst, uh remove it in its entirety and uh close the, the tongue back up again with little uh morbidity. The, the only other ones that we've encountered have been duodenal. Um And you can't excise these, you've gotta make some other plan and whether you drain it in, into the um, into the main duodenum or you drain it into some root loop or you excise the mucosa and, and drain the remnant. You've got, you've got to make some of a plan other than trying to exercise. And because I'm sure you'd end up in uh misery, I'm, I'm always concerned, uh, when the registrar says that, uh, you know, this, this needs a brilliant surgeon to be able to do his, these operations. I mean, surgery should be easy. Um And uh if it's difficult, it's probably not the right solution to the problem. Thanks. Thank you for, um, I just invite now, Doctor Mataya, who is our consultant, pediatric surgeon to make comments or share any experience knowledge. Um Yes, thanks. And thanks ga for that presentation. Um I think the, the important thing is just if you have suspicion of it, um You've actually won the case because um it's not common that you sit in any kind of differential and of off the top of your head, think it's a duplication. That's why we generally just find it in your uh because my experience, I remember it was like AAA lady in her thirties who came in presenting with um a free air which everyone thought was a duo deal of perfer. But intra op, when we actually at interop, we actually found that um she had a tubular um transverse colon uh duplication, which we actually managed to exci and actually had an, had an untoward uh recovery. But I think um suspicion is very important and then you can plan your management thereafter. But the thing that uh Doctor Maung was actually alluding to, which I actually wanted to ask um Helga about the using a linear stapler, especially with the transverse colon because we find it difficult to actually separate the duplication from the actual colon itself because it's intermittent, intermittently running or sharing the same blood supply. So the least damaging thing to the patient would definitely be to join the lumen um as was done in, in, in the case that was um shown now. And ultimately, I think that would help and it's actually it, it's actually um it it's actually uh ok, it's it's actually um Right. Ok. Sorry. No more cough. Huh? I see. You've got the entire family attending the meeting. Ok, sir. Thank you. I'll invite Doctor Moola our uh other consultant, pediatric surgeon to make any comments, Doctor Majola. Hi. Thank you for the opportunity. Nice presentation. However, and also just the cases that Doctor Mauna showed us. Yeah, my comment was basically as said, uh you wanna do minimally as you can and what is actually required for the patient. And they also do mention that obviously, depending on the size of the duplication, the noncommunicating, you create them to be communicating. And that's all that you might need to do for those patients, especially as I said, there is a closely related to uh to the structure of origin and also they share a a common blood supply. So you don't wanna uh mess with the blood supply and cause further damage um to the patient. Thank you. OK, thank you. I see Prof Delgado from is also here uh Arturo any any comments from your side, any experience I'm sure and good doctor to everyone, especially to my friend Larry. So uh I have to be honest and as I'm embarrassed to say, I have only have the opportunity in my se 77 years of pediatric surgeon to treat one patient with gastric duplication. That patient was on several years ago in Mozambique was uh 61 what the diagnosis were wrong because we took it like uh Mesenteric six because it was huge and then we did a resection and no complication after that. So that's the only common I can do and say my period on this. This is very poor. Thank you. No, that's perfect. I think you are very honest. Uh art, there is nothing wrong with that. These things are quite rare and uh and uh thank you for being honest. I see. Doctor Kulo is also here a consultant, pediatric surgeon from any comment about uh the topic. All right, thank you, bro. I'd like just to say hi to my friends from Joburg LM and for such an excellent job that we're doing that side and I want to congratulate the presenter, but other than just choose the topic, I don't have any uh comments. Thank you very much. Thank you for um I see. Um there are uh colleagues from rest of Africa also joining anybody who wishes to make any comment, please just unmute yourself and make a comment. Anybody wants to make a comment, ask a question. Do you face any difficulties? Meaning just only to bring about his history? Yes. So there's one from the rectum, there's duplication there, there was uh recognized it many, many years ago at Mido's tumor. So your a shot coming about it. OK. OK. Thank you. Um If there are no more comments, uh it is nice. We are uh ending this meeting much ahead of time. But I thank HGA for a nice uh simple presentation, a clear cut and for Doctor Mauna for participating in the meeting and sharing her two excellent cases with us. And as usual Prof Hadley and uh Prof Gado and all the colleagues, Doctor Funeka Kolo and all all the colleagues from rest of Africa. You are most welcome to join. We appreciate your presence next week. Uh There will be presentation on inflammatory bowel disease in Children and we are very glad that Professor Robin Brown from Cape Town will be our invited guest. So you will soon get um get invitation of the meeting next week. And uh I'll also put the um a link to the recording of today's meeting in case uh somebody joined late and would like to uh watch the recording again. Thank you all. Have a good evening. Thank you so much. Thank you so much, Doc take care. Thank you. Bye bye. Good evening, everyone.