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"Broncho-pulmonary foregut malformations" by Prof Sherif Emil, Montreal, Canada

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Summary

Explore intriguing pediatric surgery cases surrounding bronchopulmonary malformations in children by joining the Zoom Academic meeting of the Department of Pediatric Surgery in East London, South Africa. Led by Professor Shari Emil, Chair of Pediatric Surgery at McGill University in Montreal, this session reveals significant insights rooted in comprehensive knowledge, rich experience, and best practices from first world medical environments applied to diverse settings, including Africa. Coalesce your understanding of the disease etiology, imaging diagnoses, surgical interventions, and even possible preventive measures for this rare pediatric anomaly. Diversify your knowledge repository through shared experiences and join in on enlightening discussions that could change the pediatrics realm one case at a time.
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Description

This is an invited talk on the "Broncho Pulmonary Foregut Malformations (BPFM)" by Prof Sherif Emil, Montreal, Canada, as a part of the Zoom academic meeting of the Department of Paediatric Surgery in East London, South Africa.

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Learning objectives

1. Explain the spectrum of bronchopulmonary foregut malformations and how they might present in pediatric patients. 2. Describe the indications for treatment of bronchopulmonary foregut malformations in children, taking into account individual patient conditions and environmental factors. 3. Understand the surgical methods for resecting bronchopulmonary foregut malformations, including minimally invasive approaches. 4. Identify various complexities and challenges that may arise in the surgical management of bronchopulmonary foregut malformations. 5. Discuss the potential complications and long-term outcomes of bronchopulmonary foregut malformations in pediatric patients.
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The following transcript was generated automatically from the content and has not been checked or corrected manually.

Mhm. Ok. Um, good afternoon, good evening, good morning from wherever you are joining from. Thank you for joining the Zoom Academic meeting of the Department of Pediatric Surgery in East London, South Africa. And, uh, we are really, again thankful to Professor Shari Emil who has found out time despite of emergencies at home and at work, um, he committed, he had committed himself and he had made time to discuss some very interesting cases uh of, of bronchopulmonary malformations in Children. Um, to most of us in Africa, uh, Professor Emil, uh, is, is well known to us. He is, uh, a professor, uh and chair of pediatric surgery in mcgill University in Montreal. Uh, Canada. And, um, he originally comes from Egypt, that's where his African roots are. He studied and trained in the USA and in Canada. Um, and, and still does, uh, missions, uh, every year to Africa and, uh, and other parts of the world, he uses social media for education very effectively. That's something I have learned from him and he likes to share and he loves to teach many of us, uh, have received the donations of his book with four or three other books a couple of years ago. And we keenly read this and use this for patient management and for teaching. But above all, he is a very good personal friend, very dear to my heart. So, Sheriff, we are really thankful and I stopped sharing and you can start sharing again. Welcome. Thank you so much. Me Pine really flies. I think it's almost a year that I've joined you. But II really look forward to this and um it's just uh wonderful what you're doing um in, in um the southern part of Africa. And I know that people are benefiting from it. Uh not just in Africa but throughout the world. So really wonderful to be with you again. Let me bring up my presentation. OK. So you want me to uh start me, you um there's not a, a residence. OK. OK. Yes, thank you. OK. Very good. So I thought what today, um if we have an hour, this hopefully will take no longer than half an hour. And then, um we can maybe have some uh input discussion, sharing your experiences. I have to admit. Um a lot of the things I do here I also do in Africa, but this is one entity that II don't recall ever operating on a patient um with this type of thing in Africa. So I would be really interested to hear your experience how they present in your practice. Uh Many of them are picked up because of a lot of imaging that's done in our practice, either prenatally or postnatally. So I would really be interested in your experience because I have to say this is 11 disease and anomaly category that I really have not seen in Africa to any significant extent at all. Uh I don't have any conflict of interest. So I thought what uh some of the questions we can try to answer today are, what are bronchopulmonary foregut malformations? What is the spectrum of presentations? What are some of the indications for treatment and what are some pros and pitfalls if we're going to apply mis approaches for mis resection? The overwhelming majority in our practice are resected by uh mis approaches. But of course, um you always have to do what is uh safest for the child in your, in your environment. So, um BP FMS are congenital cystic anomalies of the neck and mediastinum. Uh There's been a little bit of a problem with nomenclature because they've been referred to by multiple entities. Bronchogenic cysts, esophageal duplications, foregut duplications, enteric duplications. In fact, as we'll see, there are uh specific features that uh both grossly when you're resecting the lesion and histologically that may make the lesion one or the other. But I really like to use the term bronchopulmonary foregut malformation to include all of these lesions. And then the histologic findings are, you know, interest academically, but they're not really, they don't really uh affect whether you're going to remove the lesion or not. So, uh I find it best to just group all these lesions under the term bronchopulmonary, foregut malformation. There is one group that does not fit under that and that's neuroenteric cysts. We're not going to talk about that today because that's a completely different um anomaly and has to do with the notochord split and so on. It really has nothing to do with the for development as much. And they're very, very, very rare. I think I may have seen one or two in my entire career. So um uh these are some of the things that that kind of allow or make surgeons use one entity or another. So again, like I said, sometimes it's gross. For example, a bronchogenic cyst can be in close proximity to the airway. It often will have cartilage um on histology and does not share a common wall with the esophagus duplication of the esophagus usually has a common wall with the esophagus. An enteric duplication is really almost d um completely a histologic diagnosis. A cyst is called enteric when you see ectopic gastric or pancreatic mucosa in it, which you don't in most of the other types of duplication, cysts in the fore gut. As we know, you can see that quite commonly in the mid gut, but not so much in the forego. So, where do these things come from? Well, we don't know for sure but the leading theory is similar to what we believe, esophageal atresia anomalies come from. It has to do with the common uh foregut ancestor of both the esophagus and the trachea. We know that the trachea is a budding off, the entire airway system is a budding off the foregut very early uh ingest creates this uh trachys fold and then these folds fuse. And finally, you have a complete separation between the esophagus and the larynges, tracheal tube, which will then continue to give bronchial buds and go on to a branch many, many times to become the lungs. So that explains most esophageal atresia anomalies. Well, and we believe that that's also the reason for this bronchopulmonary for gut malformation. And then the name itself denotes the embryology. In other words that these are sort of remnants of this budding process where you have cystic anomalies that have not joined with one or the other and are essentially left behind in the mediastinum. And I think there's strong support for that because they're all within the mediastinum. We don't really find them in other parts of the chest. Now, you could find them within the lung. That's a little bit different when you find an intrapulmonary, bronchogenic cyst. But really, these are what we're called, we're talking about mediastinal lesions. And also when we look at the histology of most of these, there is very strong evidence. This is a typical bronchogenic uh sorry uh B PFM histology where you have a muscle pattern, an inner circular muscle and outer longitudinal muscle that uh really replicates the muscle of the gi tract. There is no other muscle layers that look like that. And then often you find respiratory epithelium, you can also find squamous epithelium, but often you'll find respiratory epithelium, ciliated columnar epithelium. So you have an epithelium that is a respiratory epithelium and a muscle wall that is a gi muscle wall. And that really lends further um support for this theory that these are uh uh for gut budding um abnormalities. So how can they present well, many present incidentally, they can present through a mass effect. They can become inflamed and present acutely with fever, uh pneumonia type picture, et cetera. Uh if they have gastric mucosa, they can hemorrhage. And then there's this question of whether they um can degenerate malignantly. And then, and that's a very difficult question because the um the um literature on that is very, very, very sparse. Uh but it's not that these, you know, there can be chronic inflammation and we know that chronic inflammation often leads to dysplasia and then dysplasia can lead to cancer. But in fact, um there's very little literature to really support that and that is important uh because for asymptomatic lesions, one of the reasons we used to, you know, remove them is we were worried about that sort of dysplasia and degeneration earlier. But the, the, the literature is really quite weak on that. So that has become a bit more um controversial. So let us uh go with that background. Now travel kind of north to south in the Mediastinum, starting from, actually from the neck, going into the Mediastinum all the way down to a diaphragm and see how some of these uh can present and what should raise your suspicion that you might be dealing with one. So this is a baby um who's nine months old, turn baby, no prenatal diagnosis. But for about six months, essentially, since his parents started to give him something a bit more solid, started to have Stridor, biphasic stridor uh had had a uh outpatient laryngoscopy, uh nasopharyngoscopy, nothing really, no evidence of tracheomalacia. So observed for a while and then um had a cold and and became quite severe and actually had to be admitted to the pediatric intensive care unit. And you can see that on the soft tissue X ray, there is deviation of the trachea. So right away, you have an impression that there is an extrinsic compression or a mass effect um in the trachea. And here is a CT scan. And what you see is really uh at the lower aspect of the neck into a thoracic inlet, there is a cyst and you can see that that cyst is in the posterior mediastinum and it is associated with deviation and some stenosis of the trachea. So that gives you pretty much the diagnosis. There's not a lot of other lesions there that will behave like that. Ok. So, um this patient actually was resected uh through a thoracoscopic approach, uh very high in the thorax, but part of it was extending into a thoracic inlet. Um We couldn't get it all out by a thoracoscopy. So we opened and we did not have to make a neck incision. We ended up removing the cyst and we had a small esophageal uh tear that was repaired. We put a chest tube, got an upper gi afterwards, that was normal and the baby did very well. And if you look at the histology here, you again, exactly what I had um uh mentioned before. We have a unilocular cyst. Um there is a outer layer of skeletal muscle. So again, as you remember, the esophagus goes from skeletal muscle and about two thirds down becomes smooth muscle. So these cysts can have both smooth muscle and skeletal muscle. And so you have an outer layer of skeletal muscle and then you have an inner layer of uh smooth muscle and you have this um epithelium that's a pseudostratified ciliated epithelium. So, again, very, very typical of um a bronchopulmonary for gut malformation. Now, interestingly, um one of the things I've learned is that we don't think so much about these in neck masses in older Children. You know, when we have a neck cystic mass, we think of branchial clefts, we think of thyroglossal duct cysts, we might think of a thyroid cyst. Um We don't often think of a bronchopulmonary foregut malformation, but I've had some really interesting presentation that were kind of a surprising finding. So this is a, a girl who had immigrated from China recently, she had unilateral gynecomastia at age 10. So which she was seen by an endocrinologist who noted that she also had a significant mass in the left side of the neck. And in speaking with the family, it had actually been known to have been there for um uh over five years. So it was not something new but had been slowly growing. Uh an MRI was done and you see a large unilocular cystic mass with some compression of the airway. Interestingly, this girl was completely asymptomatic, but nevertheless, there was some compression of the airway, pretty large mass measuring about six centimeters. And we went ahead and removed that through a neck incision. And interestingly, it was actually pretty easy to remove. It was not really attached to much. It did not seem to have to share a common wall. And you can see here after we removed it, we were just curious, what was the contents and lo and behold, we get mucus. So interesting. This is a mucoid cyst here. It's a bit hard to see, but this is the bed of the lesion. It's essentially sitting between the sternocleidomastoid. And this here is the border is the inferior pole of the thyroid gland. So really completely unattached to a thyroid. We, when we went in, we thought this could be uh an intrathyroidal, um uh cyst or intrathyroidal thyroglossal duct cyst, which can happen within the thyroid as well. But the fact that it was left-sided also kind of argued against that. We thought another possibility, this could be a second branchial cleft cyst. But in fact, the histology was very clearly that of a bronchogenic cyst, an esophageal duplication essentially, but it was not very difficult at all because the common wall was very, very thin and did not have to be significantly dissected. Now, here is a patient we just presented, I don't know if any of you are attending our Global S RPS that are uh done through the DERM project uh last Wednesday of every month. So, if not, I would very, very much encourage you to join us. I think you'll really enjoy it. We do it at 8 p.m. So it's 1 p.m. your time the last Wednesday of every month. And we present cases in real time. Uh It's very interactive. It's not at all scripted. And this is a patient we actually just had in the last few months, very interesting young woman, 15 years old, um who had just immigrated from South America and had had a two year mass growing midline, but very, very low. You see where the mass is really at the sternal notch and she came to the emergency room. She had an ultrasound done before she had immigrated and ultrasound just said simple cyst. Um but she had come to the emergency room because of acute pain in the in the area. When I examined her, she was quite tense and tender in the area. There was no uh cellulitis overlying it. She was afebrile. She did not have uh any uh you know, evidence of inflammation. Her CRP was normal. Her white count was normal, but she definitely had acute symptoms. So, again, midline cyst, we weren't really sure what it will be. So we did films right away and you can see that there's a significant soft tissue swelling on the lateral film, but the chest X ray, both lateral and AP and PA are essentially normal. So we treated her with a course of antibiotics assuming that whatever cyst is there, whether it was um you know, we didn't really have adequate imaging. So we thought whether it was um a dermoid cyst in the midline, a thyroglossal duct cyst, it would still uh po potentially have been infected. And so we gave her a course of antibiotics. She actually improved quite a bit. And then we obtained an ultrasound and the ultrasound showed a very well delineated cystic mass with kind of like a fluid fluid level inside it. So that told us that the pain probably was not an infection, it probably was an acute hemorrhage or um some type of of uh intracystic uh complication because it wasn't really a simple cyst as had been described before. And you can see the label here. This was really pretty low at the sternal notch because the cyst was very well delineated. We chose not to do any further imaging. So we went ahead and this is her on the day of resection. And again, a and importantly, on the ultrasound, the thyroid was completely normal and this cyst was below the thyroid. So if you have a normal thyroid and you have a cyst below the level of the thyroid, it cannot be, by definition, it cannot be a thyroglossal duct cyst because thyroglossal duct cyst, unless you have a mediastinal thyroid, you cannot really have a uh a low uh cervical thyroglossal duct cyst, the thyro thyroglossal duct cyst is along the migration of the thyroid. And there's no reason to expect that the tract will go past where the thyroid had settled. So it didn't make sense. The radiologists impression was that this could be a dermoid cyst that has been complicated. Now, I have never seen a dermoid this large. So I thought that's a bit would be a bit unusual. In any case. We did it through a small um a transverse incision of the suprasternal notch. You see the incision here and you can see much more from this picture, but II show it to you. So here again, the incision is here, patient's head is up here, neck is down here. So it's really right at the sternal notch. And I show this picture because it was in a very interesting spot. So first of all, it was not that free, it was quite stuck. And it became clear to us that it was actually stuck to the trachea. A did not seem to be sharing a wall with the trachea, but it was quite adherent to the trachea. And a lot of it was really right below the sternal notch in the superior mediastinum. And in this area for surgeons who have been practicing for some time, you might remember a procedure we used to do and maybe it's still done. But in our practice, it's kind of become extinct because of thoracoscopy. We used to call it a mediastinoscopy. We would make a, an incision of the suprasternal notch dissect all the way down to a trachea. And then put this media stenos cope and we would do this quite commonly to stage patients with lung cancer to see if they had mediastinal nodes and biopsy those nodes. Uh So this is exactly where the location was. It was really right in the um pretracheal uh space um at the lower neck, going into mediastinum. And here we struggled with the pathologist a bit because they couldn't, you know, they, they was hard, like they couldn't really see a muscle layer. So it was hard to call it a bronchogenic uh for gut cyst, but it had a ciliated columnar epithelium and with a mucoid content. So after we discussed it, the conclusion was that this is very likely to have been a bronchopulmonary foregut malformation. So we go down a little bit now and this is a patient I had earlier in my practice in California. Very fascinating patient. A six week old girl who again had been doing very well and then suddenly developed severe Stridor had been treated as croup at an outside hospital. But because her Stridor was so severe and this felt that she had severe croup. She was given corticosteroids and she was given such a high dose of corticosteroids that it actually within a couple of days resulted in gastritis and gi bleeding and was transferred to our hospital. And when the uh gi doctor scoped her to look at the uh because of the gi bleeding, he noticed that the esophagus was quite deviated. So he left his scope in and got a chest X ray. And you can see how deviated the esophagus and the trachea are, she has an endotracheal tube that you can see here as well. Significant deviation to the right. So we went ahead and got a CT scan and you can see what's going on here. So this was a cyst, not very large, a couple of centimeters, but then this is a small baby and really right at the carina and you can see the effect here in the coronal views, you can see the effect very nicely on the sagittal views. And this is what I mean by the mass effect or extrinsic compression. And you can see again here, the cyst right behind um the tracheal bifurcation and significant narrowing of the trachea. So this case, we did by um a thoracoscopy and I have a short video here that um I'm just going to increase the volume a bit so you can hear the video. Give me a minute is just going to show you the type of dissection. This video will demonstrate a ARSC resection. Can you hear well or do you need the volume a bit higher? Uh We need volume to be increased su subsequently. Ok. Gi gi give me just a minute. I'll go even higher on the volume. This video will demonstrate it. This video will demonstrate if no is a second pulmonary forgot malformation. In a two month old girl, the patient presented with three days of significant stridor. The condition was presumptively diagnosed as croup and she was placed on intravenous steroids. She developed an upper gastrointestinal bleed and was intubated for upper endoscopy. The procedure diagnosed is steroid induced stress gastritis. The endoscopist also noted unusual angulation and some extrinsic compression of the esophagus. The scope was left in place and a chest x-ray was performed. It showed significant deviation of the scope and endotracheal tube to the right. A CT scan was subsequently performed which showed a posterior mediastinal cystic mass causing significant tracheal compression. The endotracheal tube was advanced into the right main stem bronchus to isolate the left lung and facilitate a left tosic approach. A bougie was introduced into the esophagus to clearly delineate it during the dissection. The cysts can be clearly seen in proximity to the aortic arch. The posterior pleura is open to start the immobilization of the cyst. The dissection is then carried anteriorly around the cyst margins. The cyst is intentionally punctured and decompressed of its mucinous contents. This significantly aids in the dissection particularly during its separation from the esophageal wall. The plural investments are completely divided to delineate the cyst relationships particularly to the esophagus. Traction on the cyst reveals its close relationship to the esophagus and the common wall shared by the two structures. This confirms an esophageal duplication. The esophagus stented by the boujee is clearly seen and the area of the esophageal wall containing the cyst is identified. The esophageal muscle fibers investing the cyst are carefully divided very close to the cyst wall to avoid esophageal perforation. Once the first few fibers are divided, the junction of the cyst and the esophageal wall becomes more clear and this plane of dissection is continued. The dissection is continued until the cyst is completely separated from the esophagus. The integrity of the esophagus is examined throughout the dissection. The resection bed is shown with the aortic arch and esophagus clearly seen. The patient was extubated on the first postoperative day and had an uneventful recovery. So, this is the, um, it's a pretty, uh, pretty classic way to dissect these cysts, whether you're doing it by laparoscopic or open is you really sort of have to make two walls out of one. We'll talk about that again in a minute. But I want to show you a different type of cyst that really, um, you know, it was kind of a sentinel case in my career, gave me a very different level of respect for these. So this was a one year old girl, she came in with U ri symptoms and um this was really felt to be an incidental finding a chest X ray showed uh a large uh significant mass again in the uh upper uh mediastinum. But the patient didn't really have any evidence of pneumonia symptoms were very mild and was thought that this is not related to her U ri. In fact, the U RI resolves pretty quickly and you can see again on the CT scan that this cyst is very close to the um to the uh Corina to the uh tracheal bifurcation and sitting uh completely separate um from the lung. Um So I'm going to show you uh just a couple of very short video clips that are not narrated or edited. Uh But it's important to see these, I think because it will give you a little bit of a different um perspective Um, so give me just a minute. I unfortunately could not load these on so I'm going to have to open them separately. Can you see the, the video here? Uh, uh, the video hasn't started yet? Right. Yeah. Mm. It's not playing very well. I don't know why. But anyway, um, the dissection here. Well, I'm gonna try one more time. One second. Yeah, it's not playing on the Zoom. I'm sorry. But in any case, I'm just going to go that I don't wanna belabor this. Oh, actually, now it seemed to be complain. Ok. You can see it playing right. Yes. Ok. So, you see, we're, we've dissected a, a good part of the cyst and we were down pretty much to the last few fibers. Um, and then we get into this, uh, common wall one second. OK. And you can see here that we're, we're dissecting this last part of the common wall and, um, we see a little defect, OK? And, but we're still seeing mucosa and we're thinking, ok, I wonder if we're getting into the cyst from behind and you can see that the cyst again is open. So this again, we, we do quite and, and here's a kind of junction, we're seeing some mucus there and, you know, we're thinking, OK, we're, we're just into the wall of the cyst and it's OK. And then we're seeing now a Luminal structure, OK? And we're putting our scope, this is the CCOS cope going right in. And what are we looking at? Our scope is actually in the airway. So this is a terror moment. Um on thoracoscopy, we're basically in um in the trachea. And um thankfully, we asked the anesthesiologist to very quickly uh advance the endotracheal tube into the left main stem bronchus. In this case, we were not using lung isolation cause a small baby. And we thought, you know, we just use pneumo pneumo um uh pneumothorax by insufflation and we can collapse a lung and work. Um And this baby I have to say was uh miraculously saved. You could have had a very, very bad outcome, but this um malformation was not sharing a wall with the esophagus. It was actually sharing a wall with the right main stem bronchus. So that gave me a whole different respect for this. And interestingly, I presented this at an American Academy of pediatric symposium a long time ago. And most surgeons had not seen one that actually emanates from the wall of the, of the trachea or of the uh bronchus. But of course, it makes perfect sense. If you think about it, why wouldn't it? Because just like it could be a duplication of the esophagus, it could be a duplication of the trachea. They just happened to be far less common in any case in this patient. Thankfully, she did very well. We had good and we, we just opened, we completed the cyst excision and we repaired the right main stem bronchus with sutures and she had a, a flexible bronch three months later, that showed a completely normal bronchus and she has done very, very well. And here you see cartilage and serum mucinous glands. So this is really why we uh the pathologist who often call it bronchogenic because they see the same type of tissue that comes from the bronchus again, affiliated respiratory epithelium. So let's move down now to the middle of the mediastinum. And this is a 15 year old male who is complaining of uh low back pain and was seeing a neurologist and the neurologist called me because um he discovered a mass on the MRI that he wasn't expecting. And again, it was quite typical the way it looked. And in this case, you know, if this was today, I would probably be a bit more introspective. I mean, our practice changes, I think as we accumulate cases and, and you know, again, my res my resolution to remove all of these masses has diminished quite a bit. So I think these are good cases for shared decision making. But in any case, this patient and his mom really did want this removed. He felt that maybe the symptoms are due to it. I didn't think so. He had no scoliosis though or anything else to explain the back pain. So, um I'm not gonna run a video, but I'll just show you a few of the pictures that will demonstrate the principles of resection. So here you see the cyst, the esophagus is over here and this structure draping over is the vagus nerve because it's all retropleural. So here you really need to um uh uh preserve the vagus nerve, there's no need to sacrifice it. And so the way to do it is to start by medial and lateral pleural dissection. So you dissect the pleura uh immediately to eviscerate the cyst from the retropleural space, then you dissect laterally and you can see the vagus nerve over here and then the cyst becomes really apparent. Um And again, the vagus nerve is now um is now uh displaced um anteriorly. And again, this is a really important principle. I know as surgeons, we love to remove things intact and we feel kind of defeated if we get into something when we're removing it. But these are benign and the, the sooner you decompress, the better that you will be able to understand the anatomy. And I learned that the hard way as I'll show you in a minute in one of the first ones I've done. So, once you uh again, here you see the vagus nerve now completely away, the esophagus is down here. This is the common wall. So very much like in that baby, we're dissecting fiber by fiber. OK. And here as you pull the cyst, the fibers that are shared with the esophagus start to become more obvious and you get to the very end and then you notice that we're leaving as much muscle as possible on the esophagus and just taking a very small cuff and this is the bed after removal. So there is a, an area here, it's kind of like a myotomy of sort because it's missing muscle. We do not close that um because I worry that we are going to create a scar essentially in the muscle that then could lead to stenosis. And this patient, although a very big guy was done with um 35 millimeters and then you just enlarge one of these and take the cyst out through it so very uh minimally invasive and we don't need a chest tube because we're not operating on the lung. Uh And these patients typically go home the following day after surgery. Now, this patient had a little bit of dysphasia about six months out. So I did an upper gi or esophagogram and you can see that in the area of our dissection, there's not really a diverticulum, but there's a saccular area, there's a bit of a weakness. And that is often seen after removal of these uh cysts. And that's what makes people sometimes advocate closure of the muscle. But I really worry about that. This patient I followed for several years, the symptoms actually abated and, and he's um now a university student doing very well and with exon nutrition. Now, if you go down to the media to the mid mediastinum. Again, there are, this is a nine month old baby who had a prenatal diagnosis of bilateral CPAPs. And we know that that's pretty unusual to have bilateral congenital pulmonary area malformation. So, in POSTOP imaging, he did not have bilateral CPMS. He had a CPM on the left and he had a uh two cysts in close proximity on the right to uh bronchopulmonary for gut malformations. Um The patient actually went on to have the resection of the uh left upper lobe uh by thoracoscopy. And then interestingly, it, so that was done at six months. And as he was getting into taking more and more solid food, the mother started to notice that he would sometimes gag, sometimes had a little bit of dysphasia, his weight fell off and we did an upper gi and there was definitely, again, some extrinsic compression of the esophagus. So again, we did this by thoracoscopy on the right, it had a left thoracoscopy. So we did the right thoracoscopy. And in this case, actually, it was quite easy to remove them. Again, you see the esophagus down here with the bougie in it and it was really quite easy to delineate the cyst and remove them. And again, the same type of thing we had ciliated and squamous epithelium. And again, the typical two layer muscular wall with inner circular, outer longitudinal. And now we get down all the way down to a diaphragm. And this is one of the most fascinating case I have. And it was really kind of a sobering case because it made me wonder about all these, um, cysts that we call asymptomatic and whether there's maybe something going on that we don't know until much later. So this is a six year old boy who had presented with cough and low grade fever that was not resolving over uh several days or a couple of weeks. Uh but then actually got better and um on a chest X ray, there was no pneumonia, but this um mass effect was seen that was really quite dramatic and uh a CT scan was done and you can see here this cyst with some calcifications in the wall and it's kind of like a bilobed uh cyst, a dumbbell shaped cyst does not enter the spinal column at all, but had all these calcifications. And so we were really quite concerned about the inflammation and this was one of the most difficult cases. It took about six or seven hours by thoracoscopy, but there was severe severe inflammation. The cyst was stuck to the lower lobe. Um We opened it as we usually do and the internal contents looked like there had been some old hemorrhage and calcifications and hemosiderin really evidence of long chronic inflammation in this case because it was very hard to delineate the border of the esophagus. We had the, our, one of our gi colleagues come in and put a scope in the esophagus like we do with Heller myotomies to see where um where the esophageal muscle is. So that we don't uh we don't violate the esophagus. And finally, we were able to delineate a common muscular wall and remove it. But it was a very, very, very difficult uh procedure. And in addition to having heterotopic gastric mucosa and pancreatic tissue, and that's why this one was called enteric. There was severe inflammation within wall. And that's what makes me wonder, you know, we don't really have the full picture yet. Are some of these cysts, are they going to be a problem later on? I certainly know several, the fact that they were very well known pediatric surgeon who does a lot of work in Africa who presented with this in her thirties, um with some significant symptoms due to a bronchopulmonary forgot malformation that had not been, um, been diagnosed before. And finally, um this was uh one of my early patients. Um, she was a 12 year old girl who interestingly had a prenatal diagnosis but never followed up deferred resection. And then at 12, the parents started worrying that she's now getting into adolescence and will be becoming an adult. And what if this thing causes problems? And this was at a time again where I really felt that this all should be removed. I can tell you that it's, if exact patient came to me now and I've had a couple since with a small cyst like that, I would no longer recommend removal. This was really, really, really difficult. It was at the ge junction. So half the cyst was abdominal, half the cyst was thoracic. We had to go through the hiatus to take it out. We uh this is actually the case that taught me that decompress early, do not insist on trying to remove these without um decompression. And so after we removed it, there was a defect in the esophagus, we closed it laparoscopically but it leaked and this patient was in the hospital for a month thankfully and it did fine eventually. But really, it was a struggle and she talks about her struggles very openly. She's now one of our nurse practitioners. So she didn't ended up doing very well, but it was a very sobering case. And the last thing I wanted to mention in this string of cases is that you can also have an extra thoracic bronchopulmonary for gut malformation. And this was a surprise to me again. Early in my practice, I removed this large presternal cyst and it came back as ciliated columnar epithelium where the gi muscle wall was completely extrathoracic, it was presternal outside the sternum. Um And I've had several since this is one that's a bit more recent. You can see the sternal notches here where the cyst is. But unlike the patient that I presented earlier, this was really all in the soft tissues. And, and again, you see the cyst here and again, the pathology is um exactly what you would expect from a bronchogenic cyst. So these can actually form while the chest wall is still forming and kind of be trapped outside the chest wall. So put that in your differential diagnosis when you see um kind of midline presternal lesions. So if we just maybe try to summarize all this, we talk about some of the indications for resection. Now, there is really not a lot of good literature that will guide you. And you know, uh for me now kind of getting into my, my senior pediatric surgery years, I realized that there's a lot of stuff that's just not in the books and really it's about experience. And I think what would I have wanted to know that my mentors actually never taught me or maybe just never had a case? And that's where I really tried to emphasize in any of the teachers that I do, you know in my book, etc are these nuances, what I call the art of pediatric surgery, which is really something that you cannot find in a textbook. So these are some of the things I put together. Of course, if it's symptomatic, I think that makes it quite easy. Sometimes you see evidence of significant inflammation, imaging like that patient, I just showed you with calcifications, fluid fluid levels et cetera. If the size is more than five centimeters, it's been my, my experience that most of them will uh either have some subtle symptoms or will go on to develop symptoms. So, to me, that's a pretty large lesion, but smaller than that, I think there is a possibility of observation, uh the location will influence you. If you have a small cyst at the ge junction, I would not go back now and remove it unless the patient is um is symptomatic. And do you have a secure diagnosis? Could this, for example, be a lymphatic malformation or something else that really does not need to be addressed? Um And finally, of course, most importantly, in asymptomatic patient is a shared decision making with the patient and the family. There are some pearls and pitfalls. If you're going to attempt mis resections, I do think lung isolation now we we didn't used to use it all the time, but I do think it's very helpful. It does give you more space. And if you are in this such as a case, I showed you um have a communication potentially with the bronchus and the lung is isolated, the consequences are far less significant and you can even close that by uh thoracoscopy, um I do not do them in a lateral position. Remember, these are posterior mediastinal lesions, they're in the back. So I do it in a semiprone position, um makes them much more available. Uh accessible. I like to put a large boujee in the esophagus. It really stents the esophagus and delineates it very nicely so that you don't violate it. Um divide the pleural attachments first. Um and eviscerate the cyst essentially so that you can see its borders. If there are nerve structures, the recurrent there in GL may be another important nerve that you might encounter in some of these, they really should be preserved. If um you're having a difficult dissection, endoscopy at the time of surgery can help. And and all of these actually apply whether you're doing it by minimally invasive techniques or by open techniques. I mentioned this several times. I'm really big on this decompress a cyst early and then uh carefully try to make two walls out of one. We do that a lot in pediatric surgeon, different areas of the body. There are some pitfalls do not assume that the organ of origin is the organ you will encounter. So I showed you a very difficult case of that. Um do not insist on removing the cyst in pact. Um You do not have to remove all lesions. Now, we're picking up so many of them because of a lot of cross section imaging that's done by other things. And I've now had several patients who are being observed. Um And if they continue to be asymptomatic, we we do not dissect them. These are 23 centimeter lesions. One of my senior colleagues called me in about a year or two ago because he was operating for a very small one and he couldn't find, it, just could not find it. Uh, you know, once you were in the chest, it was a big patient and very small and eventually just stopped. Um, and left it alone. Um, again, the amount of muscle, there is a common wall but you, you know, you, if you leave as much muscle on the esophagus is good. And again, I don't usually close the esophageal muscle defect, although there is some controversy about that. So I will stop here, Marland. Then II hope we have a few minutes to entertain any. I'll start looking at the chat. Um I stop my share and I'm more than happy to entertain any questions. We have several trainees from our own uh group here. So you're more than welcome to ask questions as well. Uh Yes, thank you very much. I think this was really great. Um And, and case presentations are always uh useful. Um I'll just answer your question. I don't think they are particularly uncommon in Africa. I think they're just not detected. Um And, and um in institutes where there are thoracic or cardiothoracic surgeons, uh many of them are managed by thoracic surgeons. So we pediatric surgeons don't do thoracic surgery if there is a thoracic surgeon in the hospital. So we probably don't see that often. So I think that's just to answer your uh your initial query. Uh There are interesting questions in the chat box. I think we will go uh from, from top to bottom. And uh Doctor Patankar has three questions. How often do you see cystic lesions in the neck causing airway compression? Not often. Um But um the ones that are most likely to be symptomatic, they're not common, but the ones that are most likely to be symptomatic are the neck ones because neck or superior mediastinum because that's the area where you don't have a lot of potential space. So it does not take much to get you tral deviation or tracheal compression. And often what will happen is the cyst will be there for a long time. But if it gets acutely inflamed, the pa patient just has a, this is ciliated Colom epithelium. So, just with a viral syndrome, you can have excessive secretion and then compression happens. So those are the ones that are most likely to be symptomatic versus the ones that have showed you lower down as you get closer to a diaphragm. But, um, but they're not very common. I mean, we probably see one of these maybe every couple of years. They're, they're not common. Yeah. Uh The next part of his question is how frequently does your unit see tuberculous or cold abscess or? Ok. Well, that's very fascinating because I, that we see a lot of atypical nontuberculous mycobacteria. The reason we're in the Saint Lawrence valley here in upper uh in, in Quebec, uh has uh atypical mycobacteria in the soil. And toddlers will play in the soil and then touch their mouth. It comes into their mouth and settles in the submandibular lymph nodes. And we see quite a a few atypical mycobacterial sinuses and skin necrosis that develop. But I've just seen my very first tuberculous cyst and it's really fascinating. It was a patient who had traveled to um, North Africa and came in with a cystic mass that seemed to be kind of ill defined, uh more on the chest wall but kind of deeply insinuating and we um drained it, it ulcerated became like a chronic ulcer eventually healed. I excised it and I sent it for cultures and just, this was like 67 weeks ago and, you know, they're very fastidious organisms. So just uh last week we got a PCR positive for mycobacterium tuberculosis. So that was the first one I've seen, but very, very rare. But we see a lot of atypical nontuberculous mycobacterial neck infections and they tend to be indolent and healthy kids that just give an awful, um you know, neck mass that necrosis and starts to drain and so on. I don't know if you guys see that often there. I don't recall seeing those in Africa either, but we see them quite commonly here. Uh We do see a tuberculous abscesses and occasionally them forming like a cyst like structure. Um I go to Doctor Pa's next question. What is the imaging of choice? Uh Is it CT or MRI? Either is fine. Um I mean, it depends a bit on the age because we always have to exchange the two disadvantages. The disadvantage of the CT of course, is that there's some radiation involved and, you know, we don't like to radiate patients, especially if they're very small because we don't know if they'll need more CT S in the future and so on. But by the same token, uh an MRI requires a general anesthetic in a small child, you can't just sedate a two year old or, you know, let them try to cooperate. So it's really balancing the scan, uh the the general anesthetic with the amount of radiation in general, we are going to more and more MRI S and accepting a brief anesthetic rather than radiating the child. But both of the images will give you excellent uh definition. They're both excellent in showing you what. So I don't think there's really an advantage from an imaging standpoint of one versus the other. Yeah. Thank you. Uh Doctor be Nandi who uh practices in Malawi has a question. Uh Had you bronchoscope the patient before thoracoscopy? He's uh talking about the patient where there was injury to the right main bronchus. Yeah, babe, I think that's a great um That's a great um question. No. Um for two reasons. One is it never crossed my mind. I mean, again, this is why I think it's important we share these experiences because, you know, one of my senior colleagues talks about pediatric surgery being a museum of rare diseases. And so we really have to share these experiences. I always encourage young people to publish case reports. You might think, oh, it's just a case report. But you know, I can't tell you how many times I've been kind of fortunate to find that other cases like mine have, have been reported. And so your perspective. But uh but first of all, I did not suspect. Second, I'm not really sure we would have seen anything because there was no compression of the bronchus. There was really no extrinsic compression on the imaging. I only showed you a couple of, of cuts, but the entire scan showed no compression of the bronchus, no extrinsic. Um you know, uh narrowing of the bronchus. So I'm, I'm not sure if we would have appreciated that that was coming from the bronchus. But it's certainly a good idea. You know, if you're worried that this could be, I don't think there's any problem in doing a flexible or rigid bronchoscopy before you start your THOS copy. I think that's a great idea. I think that that's a very, very good suggestion uh before you attempt uh resection, whichever way thoracoscopy are open, rather have put a scope in the esophagus, put a scope in the bronchus and then proceed I think that that's a very good uh suggestion. There's a question from Doctor Marshy. Um Do they have chance of being malignant? You have actually answered it but you can again answer. Do we have any case report on uh malignancy in uh uh B PFM? So there are some case reports in adults. Um There, I mean, again, because most of these malignancies, we are talking about a predisposition in adulthood after chronic inflammation. So there are some case reports, mahe there are very, very few. Um there really are no delineated risk factors and it's kind of like the same discussion we have about CPAPs. You know, because CPA S that are um there's this big controversy, CPAPs that are asymptomatic. One of the rationales is that, well, what if they can degenerate into malignancy or what if you're missing a malignancy? And this is a pleuropulmonary blastoma, not a CPAP again, like uh yes, there are cases like that, but I think it's hard to base a practice on it. So I have to say I've kind of removed that from the discussion. II think the chances are probably very, very low and if the cyst is just simple, no complication on imaging, no signs of inflammation, simple. Um you know, hypoechoic cyst, like most of them are and proven by cross sectional imaging, then the idea that we gotta remove this because it's going to become malignant is uh I don't think it's really supported So, can we say there is no chance? Of course not because it's never zero or 100% in medicine. But I think it's, it's uh unlikely. So, I don't really put that as part of the discussion anymore. I don't know if any of the other surgeons would be interested in, in commenting on that. But to me that's not something that's really um of concern at this point, I think, uh wouldn't malignancy happen quite late in life with, with repeated uh uh I inflammation. Uh So I think even if it happens, it will present in adulthood if cyst was left unattended and then it results in chronic inflammation, right? And you have to remember these are congenital lesions, right? So, I mean, it's not like something that developed when a child is six or seven. So if you see a seven year old or like this patient, ii, one of the patients I met a 15 year old who still has just a simple small cyst, then, you know, in 15 years, if inflammation did not occur, I it's probably not gonna happen in the next 15. So I think again, the age is very important and, and also how you follow them. We don't like to keep doing repeat. CT S as, as you get lower in the mediastinum, you cannot really follow them by ultrasound. So the idea is, are you going to keep repeating MRI? I mean, there, there's a lot of these uh nuances, I think then enter into the decision and that's why I said this, you know, it has to be uh really like a shared decision with the family on what their comfort level is. Uh I think there is a nice comment by doctor er, again, ct virtual bronchoscopy is proving to be very useful, noninvasive investigation. I think that's quite an important valid comment. Um Then I think I'll just ask, um, some of the colleagues who are here, I can recognize the ones from South Africa. I see doctor Akona Boweni, who is a consultant, pediatric surgeon at Red Cross Children's Hospital in Cape Town. And I understand she's interested in thoracic surgery. So Akona, any experience from Red Cross, any, any comment? Good afternoon prof um So we the four gu duplications that we have seen, we have two patients that were resected thoracoscopically when di was still around, but majority of them are done by the cardiothoracic um surgeons in the unit. Um So because we don't have a lot of experience in the chest, but we've had two that were resected thoracoscopically. So it is something that we are getting exposed to more and more. Ok. You know, I think that, you know, I think that's, I think that's, that's, I mean, the one, the one thing is that if you have, if you have thoracoscopic and mis techniques, I really think these patients are, you know, II II don't think doing a large thoracotomy is, is the best appro if you have those techniques available to you. And because why, because I mean, when we think about what advanced uh mis is advanced mis in my mind is something that needs significant suturing. You know, most cases if you're just going to be doing dissection, II don't really think of that as advanced mis. So in the chest uh esophageal atresia with a trach esophageal fistula. Yeah, that's really advanced mis but resection of a uh BPM, which is mostly just dissection really. I don't really see that as advanced mis. So if you have, you know, basic to midlevel mis techniques, I think these patients are excellent candidates, whether they're small or big. Um You really save them. I think significant morbidity by employing the mis approaches. Yeah. Now it's, it's hard to convince our cardiac surgery colleagues of that because they're not very mis oriented in general. There's some exceptions. Um But I think that's why, you know, probably for the best interest of the patient. If A S techniques are available, then a surgeon comfortable with that probably would be the best to remove them. Yeah. OK. Um I see doctor BN is still here. BP. Any other comments, any uh experience from Malawi? Um No, um no comments really. Um we had uh if recently we had a uh a gastric duplication, we've had uh my, my nine years in Malawi, I've probably seen about four or five abdominal duplications, but I haven't diagnosed, uh, any, uh, thoracic ones and II suspect it's, you know, uh, the ones that have, you know, minor symptoms who don't get imaging, uh, and the one that, the ones that are very sick probably get called pneumonia, uh, and, uh, never make it to us and demise. Yeah. II suspect that's, that's the reality in Africa, unfortunately. P, yeah. Ok. Thank you. Be, I see. Doctor Y cit is here. She trained at uh in Johannesburg, worked at uh Red Cross Cape Town and is in East London. Now, y anything you remember from your bar or Red Cross experience? Hi. Um I don't remember actually a single case being, being done at Barrow, but then we also did very, very limited cross sectional imaging uh at Baragwanath at the time that I was there and we did have some thoracoscopic II think that like um these cases find you once you do th thoracoscopy, you know, you think they're not there, but as soon as you have the skills they seem to find you. And we we there was even the RSC skill and I don't remember a single case of like of one of these in the time I was there, I may, I mean this was now four years ago. So. Ok, thank you. Um OK. Uh uh Doctor Nao Masha Wave is uh our consultant, junior consultant, pediatric surgeon. She also trained at uh Knox. Any experience from your side. I um thank you, prof um Thank you, uh Prof Emmo. Um I just have um a few questions. Um I'm just gonna start with a little bit. Um Do, do you have any experience with um neuroenteric cysts with your esophageal duplications? And how did you approach them? And have you ever had any intrapulmonary bronchogenic cysts as well? Um And if you did like, what was the approach to that as well? And then secondly, um I just wanted to find out what your risk factors for recurrence are. Um I see that um, obviously the aim of um, surgery is complete surgical re resection or incision. Um So what would your risk factors to recurrence be um in these cases? And um with the asymptomatic ones or the ones less than five centimeters, um, what would your surveillance protocol be? Um, if you don't obviously surgically inter um do you have a protocol regarding that or? Um, you just, I don't know if it, um And then the last question is, um, is there a role of endoscopic ultrasound? And um, do you have it available in your first world? Um because obviously we don't have it this side. Um Is there a role for it? Ok. Thank you. Those are all s super questions I might have to ask you to remind me again, but we're gonna go from the end and work backwards. Endoscopic ultrasound. Absolutely. Uh And fantastic way to image these. Um But again, if you think about um it's use in Children, it's unlikely to use it without a general anesthetic for most Children. So again, it's, you know, we consider that a, a relatively invasive technique. So if you can get a CT or an MRI without um without general anesthetic, even in a teenager, an endoscopic ultrasound would typically need heavy sedation or anesthesia. So while it's, yes, it's, it's a very good imaging technique. Um, we do not use it much. Your second question was about, uh, how we would follow it. So there is no protocol. What I typically do is if I decide that no surgery is needed, I will typically repeat one other. Um, most of the time you cannot see it on ultrasound, you know, if it's really below the neck into the middle mediastinum, the M min or below. So I'll usually do one other in 2 to 3 years. I mean, I'll see the child every year to confirm that there is no symptoms and, you know, examine the child and make sure that they really are truly staying asymptomatic. And if that's the case, I will often do another, um, final imaging before they become an adult, you know, like, could be somewhere between 15 and 17 just to make sure we haven't seen any change. And if that's the case, then that's the end of it. So I don't do annual imaging. I don't, I really just follow them symptomatically and do one final imaging before your question about recurrence. Um recurrence should honestly be close to zero. If you are not going to be able to remove the cyst completely, if I had known, for example, that in this case, um this was a really a bronchial duplication. Um Your, your other possibility is to just simply remove 95% of it and leave that little back wall, that little patch that's stuck to the bronchus. Either demise it remove the mucosa or just burn the mucosa. And the recurrence of the cyst will, it will not recur the cyst. There may be a little bit of mucus secretion for a while, but there's gonna be significant scarring and you've pretty much destroyed the mucosa. So that's your, you know, kind of like what was described before for called local cyst that you cannot remove completely. So the recurrence should really be close to, to zero pretty much. And I think you had one other question, but I can't remember it. Um Yes, profit was just your experience with cysts and um intra bronchogenic cysts as well. So, bronchogenic cysts within the lung, it really fall under the CP A category and we essentially treat them as CPAPs. There's a lot of controversy again about that. So some shared decision making, but our, our, our service believes strongly in um removing all CPAPs uh unless they're very, very small. But uh you know, we, we we don't believe in observing CPAPs and that's a big discussion. Maybe we'll have another 1 million about that at some point because that's a great topic as well. Uh, but we essentially remove them and they are not, we don't treat really treat them any differently. If you have a single unilocular cyst within the lung, we essentially treat that as a CPAP, uh, neuroenteric. I've, um, I haven't had one for over, oh, gosh, maybe 1213 years. I had one in my early practice and one soon after I came here. So the neuroenteric ones, it's the neuro that's more important than the enteric. So usually that's addressed first by the neurosurgeon. And if you can take them all out at the same time, that's great. But often you have to, to stage it and many times they present because of enlargement. And so they can present with compression symptoms, uh paresis, paralysis, et cetera. And it's really the, the neural component that is addressed first and then you can address the um enteric component afterwards. But this can be very, very, very complicated and you really need to manage them with a neurosurgeon who has some experience with intrathecal masses. Uh There is, I think one last question by Doctor Sumitra Biswas. Do you suggest MRI S and esopha esophagogastric duplication also? Yeah, absolutely. In fact, the gastric ones, uh I've had one that was a bit tricky and we ended up making a small incision because it was a pyloric duplication and it was within the gastric wall. So you really could not appreciate it. Well, but many of the gastric duplications are, are quite evident and they actually can. And um they, they'd be very amenable to MS techniques and we, you know, we're now diagnosing some of these prenatally interestingly enough. So, um the ones that are in the, in the stomach now, if you say esophagogastric, so I don't know, you know, if you mean like a ga junction one, you may actually need to do either a combined approach. If it's really going through the ge junction, you may need to do a combined approach or decide where is the largest component. And if it's best to approach is from the chest or from the stomach or from the abdomen. But I have to tell you those are the most difficult ones. If it's really esophagogastric really at the ga junction, those are the most difficult ones. And I, you know, if I'm not happy with the mis approach, I would not hesitate to, to open because you really don't want a significant leak either in the abdomen or in the chest. Ok. Uh Doctor Yashoda Man had to leave. She is second in command in our team. She has obviously thanked you. And she reminded me about a five year old child who was malnourished and had an esophageal duplication cyst in the mediastinum, which she recently resected with the help of the thoracic surgeon. So we have had a case recently. Um and, and it was done successfully. So I think that's about all sheriff. No, thank you very much. This uh really uh in addition to the interesting cases, you also had pulse and pitfalls. And I had uh we had also message and we had a wonderful question and answer station. So it was very useful. So, thank you. I will upload the video on youtube either Tomo today or tomorrow and we will think about um uh um uh CP A as our topic for next year. Wonderful. OK. Thank you so much. It's a real pleasure to be with you all. Thank you very much. You have uh a great day ahead and thank you all. Um The video will be available on the youtube and in a month's time, we are planning to get uh a gynecologist to talk to us about uh Ovarian and fallopian tube uh pathologies in Children. So hopefully she's available and, and we will have something interesting to hear from her. Well, that's great Milan cause that's an area that actually is one of my real interest and uh I may try to join you for that. But um but I think um you know, we've been talking a lot more about uh uh prenatal and baby ovarian cysts like, so that's a different, a different entity altogether. And that's I think a great one to discuss because again, a lot of controversy about what you do with ovarian cysts in newborn girls if they're diagnosed that early. Yeah. Uh, and usually the gynecologists are not involved with those babies. Yes, I still have to confirm with her, but obviously it, uh I, I'll share it on the peps a group and uh if you are free, we would love you to join. So thank you and uh we hope to see you again next month. Thank you. Bye bye Shery. Ok, bye. Thanks so much. Bye bye.