Common Neonatal Surgical Conditions, Professor Varadarajan Kalidasan, Paediatric Surgeon
Summary
This on-demand teaching session is relevant to medical professionals and is focused on neonatal surgery. Specific topics include understanding the conditions that may affect neonates, managing babies with esophageal atresia, and recognizing infant conditions that manifest as a cluster (i.e. the VAAAL complex and CHARGE). Understanding the conditions they may see is important for medical professionals to have as they are trained to provide best care and treatment for newborns.
Learning objectives
Learning Objective #1: Describe the basic anatomy of the esophagus and explain the different types of esophageal atresia.
Learning Objective #2: given a typical patient presentation, recognize whether the child is suffering from esophageal atresia or other conditions.
Learning Objective #3: Explain the surgical techniques used to repair esophageal atresia and the associated risks.
Learning Objective #4: Identify potential conditions and anomalies associated with esophageal atresia.
Learning Objective #5: Describe the diagnosis and management of bowel obstructions, including Hirschprung’s disease and atresias.
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So there are a few things I just wanted to say at the beginning. Um you will find that there are going to be some images particularly of uh some Children who are newborns. Now, these images have been taken with permission of the parents who are aware that they will be used for teaching. The only thing I would like to say is please do not share images to anyone and some images which you will see are also copyrighted and so they cannot be used for any other purpose other than for teaching. So and uh so this is a session on neonatal surgery and some of you may have attended my previous session last week on pediatric surgery and I hope you found it useful. So this is more complex and I'm not, you know, I I think I'm mainly dealing with undergraduates. So I I will not go into too much detail on the conditions, but I think it's good for you to know broadly what are the conditions that can affect neonates and the sort of basics of how we manage them. So I'm just going to share my screen now and hopefully you can see it. Yeah. Ok. So moving on. So the starting point, I wanted to say with neonatal surgery is that at least in the UK and in other countries that I worked in like Australia, what we have are Antenatal counseling clinics. And I think Antenatal counseling clinics are very useful. They are joint clinics run with an obstetrician, a neonatologist and a pediatric surgeon. And when Antenatal, we make a diagnosis of any condition, then it helps us particularly the more complex conditions to have a conversation with the parents. And so when we do that, we can advise them of what to expect and what types of procedures may be required and the longterm outcomes. Now, clearly, some conditions may have very difficult or poor longterm outcomes. For example, spina bifida with, you know, may have poor long term outcomes and some people may wish to terminate pregnancy. However, we must remember that termination of pregnancy is a personal choice. It's based on cultural religious and individuals beliefs and they should be always respected, but it's good for people to be aware of what to expect. And it's also important to know that even if you have a complex condition which is diagnosed antenatal. In most cases, the delivery can be undertaken at full term and they can have normal labor very rarely will we advise a cesarean in some conditions which may need immediate uh intensive management. Then the babies may be best delivered in tertiary centers where there is adequate neonatal and surgical care immediately available. So it avoids the necessity for transferring patients from a rural hospital to uh a tertiary center. Now, there are sometimes um you know, you may need uh earlier delivery by Cesarean. For example, there are some conditions which may be associated with polyhydramnios, et cetera, but that's quite rare. So we're going to look at it almost from top to bottom as it were. So we are going to start with the esophagus and then we're going to look at some conditions affecting the lungs. And then we are also going to be talking about some intestinal problems and then finally, some uh brief uh uh uh uh bird's eye view of uh urological conditions. So, one of the uh really challenging but most rewarding conditions that pediatric surgeons face is esophageal atresia. So this is when the esophagus has not formed normally and often the lower blind end communicates with the trachea. So you get an esophageal uh a fistula to the trachea and the upper pouch is a blind end. But over the last 60 years, the survival of these Children has improved very much. And in, in what I would say, places where there is uh advanced and appropriate care of newborns, we would expect 100% survival of these Children though they may have some difficult uh periods and may require multiple uh procedures sometimes and may require other uh care. But they all can survive unless they have other life limiting conditions. And I have said it's the ra on death row for pediatric surgeon's work by that. I mean, it's perhaps the one thing that defines a pediatric surgeon and something which most adult surgeons will not even undertake. So for example, if you repair a hernia in a baby, even uh adult uh general surgeons might attempt it. But pediatric surgeons really take pride in the work when they do conditions like esophageal tray. So this picture tells you the types of esophageal atresia. So 85% of Children who have esophageal atresia, what we call oe with tof that is tracheal fistula have this configuration where you have a blind upper pouch and the lower pouch communicates with the trachea. The others are less common where for example, you have the upper pouch, fistula, a double fistula. And this is not that uncommon, which is pure atresia with no fistulous communication at all. And then these are called h fistulas where there is no actual atresia because the esophagus is in continuity, but you get a communication through this channel. So the important thing is when you see a baby with esophageal atresia, what you'll notice is that they are not able to swallow saliva. So they'll have a lot of bubbling in the mouth and the saliva will be drooling out because they can't swallow. They might also have respiratory uh problems because they may aspirate. And so they, they may have some respiratory symptoms. So if you suspect it's a vagal atresia, then the important thing is to pass a large nasogastric tube. It's not a small one. If you pass a five French or six French nasogastric tube, because they are very flexible, they will tend to coil in the pharynx. So you must use a bigger tube like an eight French and you'll have to use it through the mouth because you won't be able to put it through the nose of a small baby. So you pass it and gently see how far it goes. Now with this seal, Atresia, the tube will stop somewhere in the knee of the upper thorax or the lower neck. And it's important not to push hard. Otherwise you can perforate the upper pouch. An x-ray will confirm this and it is very important that you examine the child completely to rule out other obvious abnormalities in the the you must have of course IV fluids with the uh dextrose or glucose because you need sugar. Babies tend to run out of sugar. They get hypoglycemic very quickly and they're often nursed slightly upright and the blind upper pouch is sucked out frequently to keep it empty. So this is a lateral view. And you can see here that the nasogastric tube is actually coiled up. I hope you can see it. And that's, and this is the shadow of the upper pouch. It's a gas filled upper pouch and you can see that the end is here. It's a very nice x-ray actually. And it's not often you get it so nicely. And what we also do is a complete x-ray of the baby. And this will help us to look for problems like uh if there are abnormal ribs, cardiac shadows and more importantly to see if there is gas in the abdomen. If there is no gas in the abdomen, then this child might have a pure atresia because there is no communication with the trachea. So air does not get into the intestine. So a child with a e esophageal atresia with no air in the abdomen might mean that they have a pure atresia. Now that is important because the approach in managing it may be different. So the other things we would do are ultrasound and an echo if it's available to rule out cardiac malformations, and then the child has to be evaluated appropriately. And of course, get and these are things which we would normally do when we get a neonate ready for surgery. So, you know, the standard uh full blood count and we always administer vitamin K to stop bleeding. And of course, they will need uh blood to be grouped and cross matched. Now, the the treatment clearly is surgical repair and the surgical repair is complex and but essentially it is about disconnecting the fistula and ligating the end and then joining the two ends. The upper and the lower end. Now, occasionally we may do a gastrostomy and the gastrostomy helps because if the repair is somewhat difficult and you are worried about whether it may stricture or you may get a leak. If you do a gastrostomy, you have the advantage that you can feed the baby through the gastrostomy. Now, if you suspect pure atresia, in the first instance, you may just do a gastrostomy because in a pure atresia, the gap between the two ends is often long and it's quite difficult to get the ends together. So what we have often done is a purely a gastrostomy in the first instance. And then as the child grows by about eight weeks, it appears that the pouches grow a little more and then you are able to do the repair. But of course, as I said, these are very complex and should be only done by people who do it quite often. It is not an operation for the occasional surgeon. And this is very important to remember when a child has an esophageal atresia, it is imperative that we look for other abnormalities. And this whole thing is called a vaal cluster. And what we mean by that is these uh abnormalities seem to go together. It is not necessarily a syndrome and you may not get everything in a particular child, but you may get more than one. So they may have vertebral anomalies in the form of hemi vertebrae they may have anorectal malformations, cardiac malformations, which could be life limiting on their own. They could have kidney problems and they could have limb problems. So they could have absence of the thumb, for example. And so you must look at the baby as a whole. In fact, if you have one of these, you must look for the others. It sounds obvious, but sometimes it's missed. And there is another association called charge. And these babies have coloboma, which as you know is a defect in the ural tract. So for example, they might have a V shaped defect in the iris. So they have that associated heart problems, anorectal problems. They may be growth retarded. So they may be smaller for their in intrauterine age. So this is another association that may be found in um Acela trai. So you can see that that is uh at the top end, one of the challenging and important problems that we meet slightly moving down further, we come to bowel obstruction and the bowel obstruction, there can be any number of causes, but some of the commonest ones are anorectal malformations where the baby may not actually have an anal opening, bowel atresia. But what we mean by atresia is that it's a blind end. So you might have a bowel, you know, where instead of being a continuous tube, the, the bit in between is fibrotic and does not exist with the lumen. So you'll have a blind end and then you'll have the rest of the bowel. Hirschprung's disease, which is the lack of parasympathetic ganglia in the bowel. So you must remember that in the intestine, the parasympathetic uh is represented by ganglion cells along the wall of the intestine. They do not exist as separate ganglia. Whereas the sympathetic chain, as you know, are in the paravertebral regions and are, are kept there as distinct ganglia. So the sympathetic innervation and ganglia are different from the parasympathetic innervation and ganglia. And of course, the condition of meconium myles where the meconium itself is so thick and viscid that it forms an obstruction. Now, commonly, this is associated with cystic fibrosis and therefore, genetically, it occurs mainly in the Caucasian population or the white population. So it is not often seen in Africa or Asia. So it can be seen occasionally but it's quite rare, but sometimes you can have Meconium mys without associated cystic fibrosis. But every child who has uh cystic fibrosis, I'm sorry, who has meconium? Mys one should look for cystic fibrosis and you can read about cystic fibrosis and how it's diagnosed, it can be diagnosed with genetic analysis and also by doing a sweat test and I have spelled congenital wrongly here and I apologize for that. So, congenital bands in the peritoneum may cause uh obstruction as well. Commonly, these bands may be associated with Meckel's diverticulum. You must remember the Meckel's diverticulum is a remnant embryologically of the Vitullo interstim duct. So I see some of you are third years and so on. So you've just about left your anatomy and embryology lectures. But vaguely, you may remember this uh structure called the Vitullo interstim duct. So the Meckel's diverticulum with or without a band is a remnant of vital Testino duct. Now, how do we manage bowel obstruction and like every other thing in medicine, you know, it all boils down to good clinical examination. It doesn't matter how much of uh uh tests that we can do and how much of imaging we can do. But at the end of the day, examining the patient and in this case, examining the newborn baby is vitally important. So you have to examine the child, make sure for example, how distended the baby is. And if there is any discoloration that you can see through the abdominal wall, because sometimes if the gut is actually uh you know, dead or dying or ischemic, it might appear blue. But remember that's only in the case of fair skinned Children, you cannot see this color change in black and uh Asian babies because the skin is too dark and you can, you will always be doing a plain x-ray of the abdomen. And then one of the things we do is a contrast enema. So we put a contrast medium through the anus when it's present clearly and we can take pictures. Now, if we see a colon, which is very small or thin and it's often the size of a pencil like that. That's what I often say. It's as thin as that. Then we are talking about what's called a micro colon and that would indicate that it has never been used. So the child has never passed meconium through it. And there are a couple of conditions where it is very diagnostic. Number one, it shows small bowel atresia or number two, meconium, myles very rarely. It can also be what we call total colonic Hirschprung's disease where the entire colon does not have parasympathetic ganglia. You could try doing washouts from below with some warm saline and see if there is any bowel movement. It may help and we can do an upper gastrointestinal contrast, but this is not always needed. In fact, a plain film with a contrast enema is often enough to give you an idea of what you're dealing with. Now, if you suspect Hirschprung's disease, because when you give rectal washouts, the baby then passes meconium, then you will do a rectal biopsy. You're looking for um, para parasympathetic ganglion cells in the rectal wall. The rectal biopsy is done with a specific forceps. It's a suction rectal biopsy. And again, it is done by people who do it regularly. So this is an x-ray of a child who presented uh with bowel obstruction and you can see the hugely dilated uh loop of small bowel hair and it's very difficult to say where which lobe this is. But because there are not too many other inter or shadows, I would suspect that this is probably a high small bowel atresia, probably a Jugal Atresia. And often when you operate, that's how it looks. So this is the dilated hugely dilated ge uh Jejunum. And here you can see how collapsed and small, the distal small bowel is and this is the arthritic missing bit. Now, the challenge for the pediatric surgeon is to animate this massively dilated bowel with this very, very thin, small bowel. And so you'll, you'll have to be quite good at doing this anastomosis. And one of the things we do is actually run some simulation, uh teaching exercises for uh pediatric surgical trainees using uh sheep or pig bow to get them to uh learn how to do this type of complex anastomosis. Now, however good, your anastomosis is one of the problems will be that the bowel may not start functioning for quite a while and it's very difficult to feed these babies because one thing you must remember is for the contents of the bowel, you move through the gut, you need opposing peristalsis. So you should have peristalsis which can close the bowel and push things through. And when you have a hugely dilated bowel, it does that but not effectively pushing things through. So sometimes it can take weeks before this joint anastomosis will work. And sometimes we may have to put the babies on total parenteral nutrition or supplemental parental nutrition while the bowel starts functioning. And this is another instance of, uh, atresia again showing very dilated proximal bowel and very collapsed distal bowel. And this is a rare condition. It's called interstim duplication. I only put it because it's of interest. And so you can actually see here, uh, that, that's one part of the interstim and this is another bit which is quite dilated. This bit is not in communication with anything and it's called interstim duplication. And depending on how long the duplicated bit is, you can actually remove it fully and join the good bits together sometimes if it's very long, it is even more challenging. And uh so that's, that's another type of reason why you might get an obstruction, duodenal atresia. Now, duo atresia is the most proximal part of the atresia that you can get in small bowel. It's to be remembered that it can be associated with Down Syndrome, which is trisomy 21. So you'll have to look for features of Down Syndrome in Children who present with duodenal atresia and often Du Atresia, I wouldn't say often, but it's not uncommon for dual atresia to have a condition called annular pancreas. So as you know, the dorsal and the ventral bits of pancreas develop separately and then they rotate and fuse. And I'm not going to go into the embryology today. Now, if that happens in a particular way, it can circle the duodenum. And so that's why it's called an annular pancreas. Now, it is not entirely agreed that the annular pancreas causes Du atresia. There is also the theory that the annular pancreas is a result of the dual atresia rather than uh it causing the dual atresia. But that doesn't matter whatever it is when you come to operate, you can't do anything with the annular pancreas. So you'll have to animate the bowel over it because if you damage the pancreas, you're going to have real problems. Now, di atresia can be diagnosed very straightforward in a plain x-ray because this is very, very typical, it's called a double bubble. So that's the bubble of the stomach and that's the bubble of the proximal duodenum. And uh you see if you see these two bubbles, then you know, for sure that you're dealing with Du Atresia, somebody marking on my slides. Thank you. Uh That is if you accidentally click as you move your mouth, OK. It's me. OK? No problem. And so that is very typical of de tray. The surgery will be to anastomose, the proximal dilated bit to the distal uh uh again, very small, collapsed, uh part of the duodenum. We mentioned this already. And Hirschprung's disease usually affects the distal part of the descending colon, the sigmoid and the rectum, but rarely, it could be a total colonic. Hirschprung's disease and we by and large, we would do a colostomy and take biopsies to find out the exact position where we have normal bowel and then come back in a few weeks to bring the normal bowel down to the rectal anorectal area and remove the abnormal bowel in between. There are different procedures for this so that you don't need to go into the details. They are all technically slightly different. They are called Swen duals or so procedures. Now, why do we have three procedures? Because not one of them works perfectly always. And whenever you have more than one operation that is tried, it means that none of them actually work extremely well, that they all have problems. So people try different techniques. If you had one perfect um method, then you would not bother trying other uh techniques. Now, sometimes you can do what is called a singlet stage pull through, which is you can take biopsies at the same time as the operation and do the pull through. But you'll have to be in a good center in the sense of you're operating on a smaller baby. And you need good pathologists who will be able to tell you quickly about where the normal levels are and you need experience. So, you know, by and large, you know, if you are not working in an environment like that and if you're not, you know, very experienced, I would suggest uh do a colostomy first and then take it from there. One of the really important principles of all surgery. But really for neonatal surgery is don't try to be clever, try to be safe. So don't try things which you think are smart, which will make you look good. Because if that is the case, you will end up having complications and you will end up damaging uh, your patients. It's better to take it sometimes step by step and do it slowly so that you have a good result in the end. And it does not matter if somebody else is very clever and they want to do something different. Good luck to them. Ok. So, uh, it, it's, it's an important condition. And so if you have anybody who is presenting with symptoms of constipation from birth, even older Children, then you must look for this. And uh, when I was working in India, uh we have had Children who are presented to us, uh with constipation from birth who have been around eight years or 10 years of age and they come with really distended abdomen and large bowel because in some parts of the world where healthcare is not as good as it should be. Sadly, Children sometimes do not present early and so always keep hash sprung in mind and you'll have to do biopsies. You can also do anorectal manometry, but that's more complex. And again, you need people to interpret it. And even if your manometry is abnormal, you will still need biopsies to prove your diagnosis. So this is another interesting condition. As you can see, this is a baby who is born with the intestines all outside the abdominal cavity, but it is covered by a membrane and you can see the attached uh umbilical cord. This condition is called exomphalos or exomes major or olo if you are an American. So it's a condition that happens because of if you remember embryology, uh around the ninth week of uh intrauterine life, the intestines herniate through the umbilicus into the sac and then around the 11th week, which is about 2 to 3 weeks later, they return to the abdomen. And of course, when they return to the abdomen, they also have a rotation which as you know, results in the actual, what I would say, permanent orientation of the bowel, the way the stomach is uh fixed, the way the duodenum curves and then the entire orientation of the large and small bowel. So you the bowel undergoes rotation when it returns to the abdomen. So this is an important thing and as I say, it happens in the first trimester. So it's very early on and sometimes uh if that doesn't happen very well, for whatever reason, you may end up with an exomphalos or olo. And for people who may be wondering why at all the bowel should come out. Uh The two s uh theoretical reasons are one is that the liver at this stage is really getting big and to accommodate the size of the liver, something has to move and it's easy for the bowel to come out of the abdomen. The second reason offered is that only by coming out and then going back in, you can have the process of rotation. So, but you know, who knows why it should happen like that? But that's the way it is. Now, how do we manage this in exomes major? The real problem here for you is that the abdominal cavity is very small because it has never had this much of intestine inside. So trying to push it all back could be quite difficult, particularly in a large exomars like this in smaller ones where we call them example as minor. It is not a big uh problem because they are just like a large umbilical hernia or hernia into the cord and they can be dealt with quite easily, but these cannot be and you cannot push it back very easily. So one of the things or very often what we do is just keep the sac nicely covered or painted with if you can paint it with whatever people have tried. So many things like alcohol, iodine, Betadine and so on. But I think it doesn't really matter what you painted with. We use uh Betadine. But if you use any iodine containing solution, you must be aware that uh you don't use too much of it because you could cause iodine toxicity in a small baby because it'll get absorbed. And then what you do is you maintain the sac in a central position and keep it all nicely dressed. And then over a period of time, it will form an, a scar and thicken and then the skin will grow over it. So you'll be left with a very large umbilical hernia and you can fix that when the child is older. And sometimes, uh in my practice, I waited till they are at least a year old. And after their first birthday, you can try fixing it when they are bigger. Even then it will be difficult. But you have some hopes that it might work. So that's now, this is a variation of the same thing. This is a condition called gastroschisis, except in this, there is no sac and the pathology seems to be slightly different because the herniation does not occur into the cord. You can see the cord here to the side of the gap, but it comes alongside the cord and is free floating in the amniotic cavity. So it is bathed in an amniotic fluid. And so one of the problems with gastroschisis is there's no membrane for you to contain or paint and leave it. So it requires more immediate attention. So by and large, we will try to reduce it and close the abdomen and you know, most of the time it will work, but clearly the abdominal pressure will go up. So sometimes you will find it's not possible to do it because it will compromise ventilation because the lungs will be compressed because the diaphragm gets pushed up. It will also cause pressure on the IVC and renal veins. So you might get, uh, you know, problems with uh Anuria or oliguria and so on. So you may then have to create an artificial sac. So you can do it with like a mesh and keep the bowel in it. And then over a period of couple of weeks, you can slowly allow it and you can slowly push it in, in stages and then close it. So, so the it, but it's a complex problem because the other issue here is that in gastroschisis, because the bowel has been floating in the amniotic fluid, it's often thick and matted. And so it doesn't start functioning very quickly. So you might have a lot of delay in uh feeding these Children. Generally gastroschisis is not associated with other major malformations. Unlike exomes in exomes, you can have associated cardiac malformations, et cetera. And they, they are more common in, in a child with exomes and in a child with exomes. The other thing to remember is it can be associated with the syndrome of macroglossia, which is a large tongue and it's called Beckwith vitamin syndrome. And these Children can become hypoglycemic very rapidly because they also have islet cell hyperplasia in the pancreas. So they secrete a lot of insulin. So one of the key things to do when you see a child with exomes is to start an intravenous drip with dextrose 10% immediately so that they do not get hypoglycemic because babies who get hypoglycemic in the perinatal period can have severe brain damage. So this is a complication of a gastroschisis which was actually delivered by Cesarean. It's one of my patients and sadly, when the Cesarean they were delivering, somehow they made a, uh, they damaged the, uh, bowel, bowel, the blood supply to the bowel. And you can see here this whole entry was, uh, denuded. It didn't exist. So, sadly, we had to remove that bit and then we had to do an anastomosis, which was quite complex in, uh, gastroschisis and the child required PN for a couple of years. But the good news is that, uh, he's now about 16 years old and doing absolutely well. So, but you can understand sometimes if you're going to do a Cesarean in a childhood exomes or, uh, gastroschisis, you have to be very, very careful that you don't damage the bowel. And that's one of the reasons we don't routinely advise, uh, Cesarean section on these Children. So this is a, this is one of the true emergencies in neonatal surgery because most of the conditions we have discussed so far and some that we will discuss soon. Actually, you do not have to operate in the first day or hour or whatever you could wait. You could assess. But this condition called malrotation is very, very uh important that you operate literally within an hour or two after diagnosis. So we spoke about rotation of the bowel. But uh sadly, sometimes when the bowel returns to the uh abdomen, it does not undergo normal rotation and normal fixation. So you end up with this situation where there's a complete rotation along the superior meric pedicle, it's almost a knot. You can see that here very, very well. And when that happens, the bowel will die and that's what's happened to this poor baby. And uh I remember this particular baby very well because it happened on the 24th of December 1 particular year. And it was a real tragedy because you can't do much with this. As you can see, the baby has lost most of the bowel. And so in these cases, sometimes we opt not to do much except remove the bowel and or even not remove the bowel and we'll have to warn the parents that the baby will not survive. Now, how do we diagnose mal rotation? The classic is bilious vomiting. So any baby with bilious vomiting in the neonatal period or bilious aspirates, you got to worry a lot and there that indicates bowel obstruction. And if you do uh an x-ray and there's not much gas in the abdominal cavity, then you suspect mald rotation and this is one area where you an upper G I contrast will help to show that the duodenum is not normally placed or to the left or the, the DJ flexure, the Duodenojejunal flexure is not to the left of the spine as it should be. And if you make that diagnosis, then the baby should be immediately resuscitated and operated within the hour or two. And anyway, if you ha if you have a high suspicion of mal rotation, then you must operate very quickly. So these are the various uh ways that the V I duct remnant can present itself. So you can have a Meckel's diverticulum, you can have a band, you can also have uh an umbilical granuloma like uh situation with a band underneath it. You can have a cyst in between and you can have a patent V I duct at the umbilicus through which sometimes you get a, a feculent discharge. So you can have all of those. So moving on, um the other important condition that we see is what's called a diaphragmatic hernia. So the diaphragm, as you know, is formed from about five different uh entities embryologically. And there is, if there is a defect in the diaphragm, then the abdominal continence will get into the chest. So for example, we said that there is this uh eventration of bowel in the ninth week of life when the, when the liver is getting big. But if there is a hole in the diaphragm, the intestines will go through that into the chest and the defect is often posto lateral defect. It's named after a chap called bole and it's a constant defect. And so you get abdominal contents in the chest, you get poor lung development, they can be diagnosed antenatal and there are actually some antenatal interventions. One can do, but I'm not going to go into that and we don't do it on all of them. The outcome will depend on how well the lungs are developed. So, and one of the complications they have is pulmonary hypertension when they have poor lung development. So this is a um an older child actually for I think this child had some problems. I it's not one of my patients, but I came across this x-ray, which shows that the stomach is actually in the chest. And this is a variation of uh diaphragmatic hernia. This is called an A ation. So this is this is where the diaphragm in one part lacks muscle. So it's a membrane rather than a muscle because you must remember the diaphragm is a muscular structure. So instead of a muscle, if it's just membrane, then the stomach can even right up into the uh chest. So management of diaphragmatic hernia. Well, if you see a child with diaphragmatic hernia, which you were diagnose by taking a plane film and you will see that the intestines are in the chest, you could try to put an NG tube to keep the stomach empty and stop the gut distending with that, they will require intubation and ventilation. But you must be careful. We prefer low pressure ventilation. So there is a type of ventilation called oscillation, which is better than positive pressure ventilation and there is no hurry to operate. And uh about 40 years ago, people used to operate on them very rapidly. And around the time that I started training in pediatric surgery, it was realized that immediate surgery does not really help. And what actually improves outcome is to stabilize the baby and then operate even sometimes we operated even a week later. And so you need a stable baby who is well ventilated, well compensated in every way and physiologically as near normal as possible. And that seems to improve the outcome. But this is one condition even today in the really bad diaphragmatic hernias, you have a mortality rate of about 25 to 30% anywhere in the world. And so, you know, which is, which is that group which we may attempt in uteral intervention. Now, surgery uh is clearly not that complex. Actually, it is to return the bowel to the abdomen and repair the defect in the diaphragm, which can usually be just sutured. Sometimes we may have to use a mesh to bri bridge the gap. So we move on to anorectal malformations. So this is where you do not have a normal anal opening. And the the word of warning is even if somebody says that the child has passed meconium. You have to look at the opening because sometimes the the opening is abnormal and you can pass meconium through an abnormal opening. And similarly, the presence of an anal opening does not mean that everything is normal because there is a condition called rectal atresia where the rectum is absent. So you look as though from the outside that the anus is normal. And this is because embryologically, you remember that the anus develops from ectoderm and part of it also develops from the derm inside and you get the junction of both. So you can have the external uh part alone. So, and you must always look for other malformations. I'm not going to go into this in great detail. And uh so you have different types, they're called high or low depending on how high the atresia is. And this is a male malformation where you can see that the fistula is to the urethra. So you get a recto urethral fistula. So most anorectal malformations anyway, from your point of view, are managed, unless they have a low variety, they are managed by doing a colostomy initially, then doing a good assessment of the anatomy and then doing surgery. And the commonest procedure is called the posterior sagittal anorectal S. So this is an x-ray which shows that fistula actually very well. So you can see the bowel, we put contrast through the stoma colostomy and you can see it has gone into the urethra there and into the uh bladder. So females can have a fistula into the vestibule or vagina. So you can get rectovaginal or recto vestibular fistula. And this is why passage of meconium does not mean everything is normal because you can actually have meconium in the, in the perineum, but it may be through the fistula. There's a very rare variations and one of the rarest and most complex ones is called cloacal malformation where there is only one opening uh in the perineum. So this is how it looks. So you will see that the bladder, the uterus and the bowel all open through one channel. And it's uh it's exactly like a bird actually. And this is a very complex malformation. And again, the initial management will be a colostomy and then really should be reconstructed by people who, who do it a long time. And uh so it's not for the casual you occasional surgeon who does anorectal malformations because you get only one chance to get this done properly because you have to have a urethra, you have to create a rectum and you may have to use skin flaps to create a vagina. So we have had a few of those over the years and my oldest patient, which I did on my own was uh is now about 24 years old and she's doing reasonably well. So here we're just going to mention that you could have uh congenital malformations of the lungs. They are got different names, but they all often present as cysts or uh you know, emphysematous uh changes in the lung. So, pulmonary sequestrations do not have normal bronchial communications and they can present at birth with respiratory distress. So this is just to show there are two types of sequestrations, one which have a complete pleural covering and they are called intralobar. So this is the sequestered lung and the other where they have two separate plural coverings. And this is called extralobar. They can present with respiratory uh distress or dyspnea and tachypnea. And sometimes they come with recurrent respiratory infections which occurs in the same lobe. So if you see an older child who seems to get pneumonia in the same lobe all the time, you must suspect sequestration. I'm going to, I'm not going to talk about those. And so the, the way we manage the sequestration is to remove the uh affected lobe, do a lobectomy. But one should have a high degree of suspicion. As I said, if somebody has recurrent infections and uh this is called CCM congenital uh cystic adenoid malformations or nowadays, they go by name, Pas and CPA MS, et cetera. People like to change the name makes them feel good. But essentially this is the pathology. So you get uh you know, lobes which are replaced by cysts and pseudostratified epithelium. You might also get uh cartilage and muscle in the lung. So these are really abnormal uh parts, I mean abnormal lungs and they are classified depending on the size of the cyst. They can be associated with these other conditions. And as we have said repeatedly, if you find one abnormal condition, you have to look for others. And sometimes if the CCM is really bad, it is not uh possible to sustain life because then the PM, the actual good lungs are very hypoplastic. The child has uh polyhydramnios. Sometimes it's uh associated with hydrops, fetal, they can be diagnosed antenatal or later on. As we said, if you have recurrent infection treatment, I mean, the diagnosis is with plain films, ultrasound, not that good, but can be used. CT and MRI may be useful bronchogenic cysts and these are actually four gut cysts. And so they, they are formed in association either with the bronchi or with the esophagus. And sometimes peculiarly, you can find uh cysts in the tongue or pancreas which are lined by these typical epithelium or which you will find in the bronchus. And we can't really explain how that happens. Ok. The treatment is of course, cyst and Lobectomy. And remember a lot of this now can be done thoracoscopic where the facilities are available, if not available, you'll have to do it by open uh thoracotomy, emphysema, you can have lobar emphysema and it's due to lack of cartilage. So the lungs will expand, but the bronchi, particularly the smaller bronchi if they do not have adequate cartilage. When the chest collapses in uh respiration during expiration, they collapse too. So there is trapping of air which causes the lobes to expand. And so people have done research on this and there was a pathologist in Melbourne who did a lot of work on this and they showed that these uh lobes have a diminished amount of cartilage. But the treatment is very similar, you will have to do a lobectomy. This is a very good uh example of an emphysema as lobe, which is actually herniating to the other side, to the opposite side. And you can see there is a mediastinal shift. So sometimes you'll have to do surgery on them quite uh quickly. So coming to the urology bit, I'm just going to touch upon two or three quick conditions. This is ectopia ves. So this is where the bladder is completely open. It's called bladder exstrophy. You don't see this very often and they are quite rare, but they are quite a difficult condition to manage. And if you diagnose or see them, they have to be referred to a specialist unit. In fact, surgery for uh bladder exstrophy in the United Kingdom happens only in about three or four centers. So for example, I haven't done one in years because all our patients from Brighton go to London because they are so complex and so rare. We decided about 25 years ago in the UK as pediatric surgeons that we would centralize it to about four centers across the country. So they are the only ones who deal with it. And they can be quite a devastating problem from the point of view of continence. And in boys, from the point of view of their sexual function later on. And this is a variation of ectopia S A where you can see that the bladder is not fully open, but the urethra is fully open. And so often it's a uh a whole range of uh uh associated problems. So the hypospadias is the opposite of this where the opening is not in the normal position or, but on the undersurface of the penis. But there is one particular uh problem that I want you to be aware of. If you see a baby with a very severe hypospadias where the opening is here and the phallus is there. And you will think this could be a penoscrotal hypospadias and it looks like a scrotum. You have to examine for the testis because if you can't find normal testes, you have to think of a condition called congenital adrenal hyperplasia, which is a vir realizing condition. And it's because if you go to study you the uh pathway for formation of steroids, you will understand that unless you have the correct enzymes, you will not form normal steroids. And if you don't, you will end up producing uh vir realizing hormones which can then result in a baby like this who looks as though this baby looks as though it's a boy with undescended testis and uh hypospadias, but it this child was actually a girl. So you have to be very careful because if at birth, you wrongly labeled this child as a girl or I'm sorry, wrongly labeled it as a boy with hypospadias and undescended testes. You have a real problem because you are completely giving the wrong gender to the parents. So this is a sort of urgent situation at birth. So any child where you cannot feel the testes and who has hypospadias, you have to rule out the possibility of a virilize syndrome like a adrenal hyperplasia. So please remember that. And of course, we can have uh babies who have antenatal diagnosed hydronephrosis like this child where you can see you a very hydronephrotic kidney and that may be due to various causes. One of the conditions is posterior urethral valves and that's because of abnormal valves at the junction of the posterior anterior urethra, which you can diagnose here by putting contrast and it's called an MC UG or maturating cystourethrogram. And uh you'll find a dilated elongated posture, urethra. The way that you should manage this, you know, unless you're a specialist urologist is to just pass a urethral catheter or a feeding tube through the urethra into the bladder so that you drain the bladder and then you need to refer the baby to uh a proper pediatric urology center and they will often present with, uh, not passing urine with a distended bladder as a unit or with a dribbling of constant dribbling, uh, small drops of urine and you'll be able to palpate a big bladder. The outcome of this will depend on how much the kidneys have been damaged even before birth. So, you know, that's one of the problems we have that they could have back pressure on the kidneys and the kidneys could be badly damaged even before birth. And the other condition that you can diagnose is reflux. Here, you can see vesicoureteral reflux and quite an abnormal looking bladder actually. And uh so reflux is another problem that may cause antenatal hydronephrosis. And if you have a child with reflux, you must start them on uh prophylactic antibiotic and then refer them to uh pediatric urologist. The commonest prophylactic antibiotic we use is trimethoprim and this is just to show how you manage them. Sometimes you have what's called perinatal torsion. And this is a child who was born with a dead testis at birth. And this is often happens uh when the testis is descending in the third trimester and you can't do much to salvage that testis most often they are completely dead. So this is the final slide. I think one of the principles uh or the main principles of managing Children with surgical problems is to make sure they get good. Uh hydration, glucose is important keep the baby warm. So, hypoglycemia, hypothermia are extremely bad for new bones and they have to be well oxygenated, sometimes post-operatively, nutrition is very important. And so you may have to resort to PN use antibiotics appropriately and you may be having to do with temporizing procedures. I have not touched upon one particular problem because it's called neonatal necrotizing enterocolitis. The reason why I haven't touched upon it is I would suggest that most of those babies are managed, uh, medically and the few who develop necrotizing enterocolitis may come to surgery though, I think many of them will have surgical input. And, uh, so that's one that's a huge topic by itself. And as you can see, we have had to cover quite a lot. So, thank you very much and has anybody, I'll just look at the chat to see if, um, there are any questions. So a has raised his hand. I don't know if you'd like that. Yes. Uh, yes, it was regarding cyst. Uh, I wanted to see a slide, uh, before the cyst one, the one where the treatment was the, uh bronchiogenic cyst. Yes. So there was a slide before the treatment of the cyst, which I didn't to see properly. Which one? This one, uh, it was about treatment of the cyst. Let's see. Ok. This is to do with cyst. Ok. Uh, ok, I have, I have got it. Thank you. So you have it now. Yeah. Yes, sir. Yes, sir. Thank you. That's fine. Um Let me see. There was something else on the, well, somebody had asked about, I just missed it. According to my level of attention, it is gastroschisis plus hypoglycemia. It's, it's Danya Beckett. I'm not able to understand that question. Do you want to ex my question was, you said gastroschisis is associated with the syndrome with macroglossia and insulin and um, islet cell hyperplasia. What was the name of the syndrome? No, no, no, it's not gastroschisis. I I said om is associated with macroglossia and eyelet syndrome, not gastroschisis and the syndrome is called Beckwith vitamin syndrome. I'll put it on the chart. Ok. Thank you. Um Hiba just has one last question. But if I'm able to end the recording and you could answer that one after the recording's ended, I just take a moment to thank you for your time today and to remind everyone the next lecture is starting in a couple of minutes. Thank you very much. Somebody is asked about interstim or transplant.