Super Bowl Grand Rounds (10/4/2023)

Thank you. Good morning. One, one awake. We do those experiments this morning. And I'm very optimistic about it, but all experiments are experiments. When I was a medical student, I know most of you weren't born yet, in the early 1980s. Grand Rounds at the Children's Hospital, always a medical student at Chicago, which used to be called Children's Memorial, Grand Rounds was the entire hospital. Everybody went to Grand Rounds. Now, with simpler times, institutions were smaller. There was less hyper-specialization. And I just thought that was normal, right? And everybody learned from everybody else. And all the different disciplines were together, and immunologists and cardiac surgeons and psychiatrists. That obviously is not a practical thing to happen in this day and age. But it really was interesting to learn from each other. And so much of what we do, at least my own personal experience, I've learned from people who are not in my discipline of general pediatric surgery. And I still do. The things I do today, I was taught by orthopedic surgeons and plastic surgeons and dermatologists and hematologists and radiologists. I have had people, nurses, no, come. I've had people come and scrub with me for decades. And, you know, Jim Kassler taught me how to use a tourniquet. And John Mulligan taught me how to do tissue advancement. And I stopped the mere beginning in the hall a couple weeks ago for advice on how to handle a case. But we tend to have our academic meetings in isolation. So when I became surgeon and chief, I had a concept of let's try this Super Bowl Grand Rounds. And see if we can get everybody who works in the procedural world writ large together, a couple of times a year to learn from each other. Now a few things got in the way like a global pandemic, some sort of urgent situations in the operating room. And so we used the times that we'd scheduled for that to talk about how to manage restarting the OIRs, how to pandemic and how to deal with the fact that we didn't exactly have all the instruments we needed at the right place at the right time. But now I want to try it. So I have sent out to all the chiefs a couple of times a call for cool cases. What would you and your specialty think is a cool case that everybody like to hear about? Hopefully interdisciplinary so that multiple people can show how they worked together. I got lots of responses, lots of good responses. And we're going to we have two days a year on our base calendar to do this going forward. And so we'll see how this goes and see how people respond to it. So today we're going to have a couple of cases. The first one, I don't know which one, the test well in second, right? That one was actually suggested by my Pedro Del Nito. And the first one has to be presented. So many of us saw it was very dramatic more recently that it was kind of obvious. There are several others in the wings of people who today wouldn't work for. So we're going to we're going to have Benzende Haas lead this. Most grand rounds we start by introducing people formally and say, well, they went to medical school and you know, all their honors and all that. We're not going to take the time to do that because if I did it for Ben, it would take too long and if he did it for everybody else, we would have no cases to talk about. So I'm just going to let Ben take the station to their base. I'm going to say Benzende Haas is a pediatric general surgeon who is a really good guy and most of you know. Okay. Okay. We got started with the first case. And I'm going to invite my colleagues to come out work. I'm going to invite Dr. Decker Fas, Dr. No, Dr. Quann, Sonia, Ben, up here already and Christine. Please join me. And this first case, there we're going to be talking about. Choose a, it's going to be going to play musical chairs as well. This first case relates to the emergent management of a child who came to us with a light threatening bleeding event. And so at the time we did not know that was the case related to a bun battery in just. Okay. So we're going to start off. Sonia, tell us what did the ER know about the situation. Great. Just testing everyone can hear me. Okay. Hi, I'm Sonia, one of the ER fellows, but attending at the time. So this was a busy Friday afternoon in April. And I got a call as a medical command at about 430 PM that there was a 13. Month old male who's at BMC at the time that was coming in for a button battery ingestion. So there was concern for upper GI bleeding at the time. This very brief story that I received was that he had been seen a few days ago for a viral type process. But then unfortunately he came in looking much worse and they saw. You know, epistaxes some. And they saw a button battery on a brief chest X ray that was in the esophagus, which was described. Since it's a very short turnaround, we couldn't actually get the images very quickly. They also had seen in the few days span, pardon me, a hemoglobin drop of from 9 to approximately 5. I know you probably turned the chromaticrit more, but they had started a transfusion at BMC. So thankfully somehow this kid was still on room air. Blood pressures were, you know, stable at the time. And the critical care transport team from Boston Children's was already on route as I was getting the call from our communication center. Next slide. So when I heard the story was already quite nervous and thought that this kid was definitely a critically ill. And so I had the communication center, which is amazing. I try to reach out to many of the specialty teams here, including the general surgery team, which is on our pathway for button battery and ingestions, ORL and also GI. We also at the time had just notified like the main OR to try to give them a very brief heads up. And of course, at this time, there was a question in my head from the ER, could this kid with a life threatening a button battery ingestion go directly to the OR. We had very limited time and very limited information other than the brief story. So after a short discussion with the surgical teams, it was decided that he would go to the ER first. And with that knowledge that this was probably a life threatening bleed, I prepared to make sure it would be an oral stat when he arrived. And then our ER priorities really were for airway protection, although I know this was a very critical airway. Transusion was also on the list and then disposition from my end was really to try to get him to the OR as quickly as possible. So brief time course, we got the call at 430 and within less than an hour, he was already in the ER. We'll go over his exam briefly, but he was in extremist when he showed up. All the surgical teams were already thankfully very, I think they were already involved in a trauma stat in the ER at the time. And so many people were already there. When the patient arrived, we activated the massive transfusion protocol right away. And he came in with one foot PIV, so that was a bit stressful and we had to work on some access. And lo and behold, he also happened to have to generalize tonic chronic seizures in the ER at the time. They were brief, but it was very, you know, whether it was provoked by systemic illness or stress verdict is still out. And then shortly after we just transferred him directly to the OR. Just to summarize his exam, he was just in extremist with, you know, file science that reflected probably the blood loss and respiratory distress at the time. Okay, the sequence of events for nursing was that the charge nurse was alerted via the trauma pager that there was a button battery ingestion that was coming up from the ED. We knew that involved doctors in day house GI or L and cardiac, but that was really the only information that we had. Chris Benson relayed that information to the staff and we quickly got a trauma team assembled of people with very clear and defined roles. Alicia Hamelberg was a resource for the room. She was able to go up to GPU to get the GI scope. She helps set up the bronc table. Somebody from GPU was able to come down and assist doctor know with his portion. Katie Lucy was the circulator. She got report from the ED. She coordinated with the blood bank and she assisted anesthesia. I was scrubbed ready for a sternonomy and any vascular or esophageal work that needed to be done. Sierra Bambachi stayed with me. She helped me open the case count and get whatever resource whatever I needed. We were very fortunate that day that we had enough staff and that they were very experienced in this multi disciplinary approach. We got the RAS at 5.15 pm. It was a very busy Friday afternoon. Usually during that time of the day, the team is limited to the call team. We start narrowing down the team. We didn't have enough information about the kid. All we knew is that there was a bleeding patient in the upper and the ED that required emergent or assistant. We gathered around pretty quickly a team of five anesthesiologist. We had the resident NSERNA to set up a room. Again, we didn't know what to expect, but we were expecting the worst in terms of like difficulty of the airways. We had all the equipment that we needed a video learning scope, a direct fiber object. We were ready to set up for more lines. We gathered the medications, the pressures, more blood products and warming devices. One of our colleagues went to the ED to assess the severity of the situation and to assist in any airway support as needed. We hear back from our colleague that the kid is critically stable. He's fairly tacky, cardiac and tachypnic, but he can maintain his airway. We decided to expedite the transport to the operating room in order to intubate him and more of the control setting where all the teams were available. We had the right equipment, the right team and most importantly, we were ready to intervene in case he crashed on induction. We got in the room at 5.31 pm. It was a very well orchestrated multi-team induction whereby some people were working on the airway that was luckily easy using the video learning scope. Others were aligning up the kid because we anticipated the need for blood transfusions. The PIVs, the surgical team inserted the triple-loom in central line, some were inducing and giving blood with induction along with some pressures, some were checking the blood and getting more blood products. The challenges for out-sour out-the-case were the risk of losing the airway. It was very crowded at the head of the bed. The GI was scoping throughout. It was a smaller kid with a small tube. There was a risk of airway displodgment. When we got into the massive arterial bleeder, the face was full of blood and there was a risk also of airway displodgment along with hemodynamic instability. We had to intervene pretty fast. We did transfuse massively and we were running into the risk of herulopathy. During that time, a lot was happening as you can tell. The only time when we ran into reading with dynamic instability was where we removed the clot and we got into the massive arterial bleeder. Luckily with all the teams intervening at the right time, we got him up in no time and he spent less than two minutes in lower blood pressures. Another thing to highlight is the amount of blood products that the kid received just in summary. He did receive an equivalent of one blood volume in fact, since along with many other blood products. From the general surgery standpoint, we had the luxury of having several colleagues available. The actual surgeon of the day was Dr. Zolekis. She heard about this patient and started things in motion. But at the same time, there was another life-threatening emergency in the cardiac cath lab that she had to go and perform an operation there in the cath lab. Dr. Dickey, who was the ICU rounder, was in the trauma bay when this patient arrived at night, was a nosy bystander. They had heard about the situation from one of our colleagues at B&C who happened to call and say, hey, there's a child with the situation heading your way. It was happened to be Friday afternoon and it had been done in my case. I was about to head home. So I just wandered over the ER to see what was going on and see if I could help. Unfortunately, the extra hands on board from all the departments who will hear really was helpful. Peter, no from GI. I happened to be on that weekend and evening and it was in my office and I was also on our advanced GI and us to be service team and on as well for that service. When this patient came, we knew the proximal location of this button battery already and we also knew that there had been with hematemosis and hematocasia, likely a large arterio-osophageal bleed. And we had designed already a kit to deal with this issue in small pediatric patients. This is a 13 month old and adult tamponod devices for varices are just not appropriately sized for a 13 month old. They wouldn't work. So we had already made this kit. This kit was brought to the emergency room and when the patient went to the OR, we had, we could tell that the vitals had stabilized. There was dried blood around the nose. The belly was still soft. So there wasn't an active bleed at the time, which gave us time to set things up. But we knew there had been a sentinel bleed, which is often the case before larger hemorrhages, which are in many cases with button batteries fatal, even when they occur in medical settings. So that gave us time to set up this tamponod device and then after that, we started with the endoscopy. And with the scope first entering into the esophagus, we saw a large clot throughout the entire length of the esophagus filling most of the stomach as well. It is likely that the clot had tamponodid de bleed itself. But then this gave us time because we did not see active bleeding to advance a guide wire down in an oral gastric guide wire into the stomach. And here you can see, we have both the guide wire down clamped at the bite block, the tamponod balloon up against the bite block, ready to be used piece of pink tape on the sheath of the tamponod balloon in order to estimate depth of insertion and importantly, the patient is prepped and draped ready for sternotomy. The team is already as well and patients receiving blood products has all their appropriate access and the airway secured. So then we focus on the balloon. You can see just the edge, sorry, the battery. The edge of the battery is visible right there and the guide wire is just visible off to the side as well. So we knew with removal of this battery, there was a good chance of restarting the bleeding, but the battery had to come out. The battery was in there pretty firmly. We after a couple of tugs, we were able to get out. We did not have immediate bleeding. That allowed us to change a scope to a larger therapeutic scope, wash the area, improve our visualization, even apply the acetic acid, which is recommended in these situations to neutralize the alcohol and environment that the battery creates to prevent ongoing tissue injury. But then we had these two large clots in the upper esophagus, a big two centimeter by two centimeter. Ulceration covers about 75% of the posterior wall and the size of the esophagus there. So we suspected carotid artery involvement just from the location, but then we had a team pause. So we stopped everyone and decided, okay, what's the best thing to do for this patient? Are we all prepared? Is the surgical team prepared? If we take off these clots, there's a good chance that bleeding is going to occur. But if we don't take off the clots knowing there was a Sentinel bleed, it's a Friday evening, even if you go back up to the ICU of one of these pops open in an hour or two or later in the evening, the ability to get there and manage it appropriately would be limited. So we decided, let's take them off. So we started with the right clot, took off the right clot. It was a pretty clean base, no active bleeding from there. So then we went to take off the left clot and just massive hemorrhage. The scope came out followed immediately by a rush of blood into the mouth and out the mouth and the tamponade balloon then went into the appropriate depth that we had estimated blew up the balloon and that pretty much stopped the bleeding. And we had to balloon up for the entire duration of the surgical intervention to reach the crotted at a brief period when the crotted was reached, we took the balloon down after about 24 minutes. And even at that point in time, just rapid rush of blood out of the mouth and again a drop in the a line pressures and we just reinflated the balloon long enough so that the team surgical team could get control and clamp off the vessels. I'm Michael Quant from cardiac surgery. So the key question in our minds when we first got the call from the ED about this patient was where is the bleeding coming from the options include the crotted arteries branches of the thyracircicol trunk which feed the upper third of the esophagus. But more importantly and even more recently the aortic arch so you can see in the diagram on the right side that there is relatively close opposition of the distal posterior aortic arch to the lower part of the upper third of the esophagus so that even if the button battery appeared on the chest x-ray to be somewhat higher up, it is not inconceivable that the injury could be there and that's described in literature. We considered there for a CT scan but that was when the kid was seizing in the ED so we abandoned that idea and we went straight to the operating room and our plan was to have an extremely low threshold to perform a definitive sternotomy primarily so that we could get immediate control of the head vessels. As well as to allow for the possibility of going on cardiopony bypass and using circulatory arrest if indeed the injury was at the level of the arch which would require a circulatory arrest for repair and so it was key that we knew from doctor know that the injury was on the left side. So it helped us to prepare for the incision that you see on the right side diagram. We positioned the patient supine with the shoulder roll and we had perfusion and the cardiac OR team in addition to the general surgery team at the bedside with turnipids and cannulas and everything ready in case we needed to use bypass. And in addition to just having the field prepped prior to manipulation of the battery were scrubbed in and ready to go before the battery was actually pulled out. When we saw the bleeding we made the incision we were able to get vessel loops around the bases of the enominant artery and the left carotid artery within about two to three minutes. But by that time doctor know had gained control of the bleeding with with the balloon. So we had the luxury of time to then dissect out doctors and DS and I the fistula which was hard to do with the large flag mon in place and the and the button sorry the balloon inflated which created a lot of distortion of the anatomy. So when he let down the balloon we then hepernize and clamp the vessels to control the left carotid that allowed us to then have control of bleeding while at the same time understanding the anatomy well enough to then perform this deception where we then took down the fistula, debrided the carotid and that's the resulting hole that you see there. We were then able to place a bovine patch of pericardium on that area to reconstruct it and then doctors and they has I believe primarily repaired the esophagus and a little tough to see the actual edges but you get a sense of similar size perforation in the esophagus just on the left-sided yellow circle. He fortunately did very very well after all of this. This was very dramatic that he we kept him asleep and then the ICU for a few days remember cover after this ten years of the soft year repair. And he was in the hospital a little less than a month. We did several endoscopic surveillance evaluations of this area that either week it looked really good that we expected this district or down so we continued our endoscopic surveillance. He's had a few violations after that and looks even better now. A important thing you know he's not eating everything by mouth without even a YouTube no neurologic sequela so excellent outcome overall. He did because of the dissection and the involvement of the fish that are right through the area with a left left recurrent orange nervous that was I think affected but fortunately he's able to compensate and is not aspirating. So obviously this is a case where all the stars really aligned we had despite being a Friday afternoon everybody getting ready to go home. There was an abundance of resources expertise attitude communication that went amazing and we want to take a few minutes to reflect on these elements as each of us saw those. So just very briefly from the ER perspective this was a very challenging case in the sense that we had limited information and limited time before the patient arrived. The family was also patient Creole speaking primarily so I think the language barriers just in the case itself probably made it made it a very tough thing for the family to see happen both in the ER and as we emergency left. I think things that I thought from the ER perspective we could have tried to reach out to blood bank even earlier potentially and of course we had multiple calls with the surgical teams but perhaps now with the effort to do a zoom call for discussions for our transfer. From our standpoint I think we had enough people on a Friday afternoon which had pretty much everybody had everybody we communicated pretty well we set up the room pretty quickly and everybody helped us get the equipment that we needed. What we can do better just if we had more information but again it was pretty much an emergency and we didn't have enough time to talk to the parents to the family and communicate with them the severity and the acuity of the situation. It's not immediately apparent that cardiac surgery should be called in situations like this but the more you think about it it becomes quite obvious and so I think the team did a really good job letting us know early. We didn't get the call when the patient was necessarily en route so that's one point of potential improvement to work into the protocol but nevertheless as soon as the patient arrived we were notified immediately. The improvements that we've made for nursing are that we have two of these emergency a staff of geotamp not kits that live on the emergency cart in the operating room. The staff has access to the GPU to be able to get scopes off hours if need be and Alicia and I are planning for an in service for the staff for education for this type of emergency case. So so many things went well from the from the perspective of the entire team and how prepared everyone was and so there's so much teamwork there but there was also teamwork in getting these kits and the whole process it was a several year process this is what we have currently we have two of those kits there on the crash card there's only one in the picture because one is actually up at a patient's room right now but we had how did we get to from this idea in like 2020 that hey the adult devices won't work for a 13 years. So we had come up with this idea to use this the soft deal tampon on balloon it's a pretty rudimentary just a manual inflation syringe but then that's what originally in 2020 and 2021 we had brought up to the I see we got some extra input and changed it and now we have this simplified device we have a location at home for it plans for keeping it updated and preventing it from expiring and now also video with a QR code for point of care educational instructions right on the on the kit is that QR code should be coming so that was a lot of teamwork all along the way as well and a lot of input that led to it getting to there and being successful use. Perfect so the summarized this case I think it's a great example of the right people the right place with the right resources with excellent teamwork at the several there were not up here that are in the room really I want to thank you everybody went above and beyond on a Friday evening we had clear rules we talked about contingency plans about what could go wrong we had shared decision making you all had the same mental model and it was really as I think back on it it was really important to have a fairly quiet environment despite 2030 people in the room it felt like we all had a good understanding of what was happening it was in chaos and it was a smooth resuscitation treatment and recovery for this child because of that so next time as I think about this is like we know what works we know what resources we need let's try to replicate facilitate and encourage and recognize when this sort of things happen. We will continue to work on trying to fine tune a few of these elements we talked about for example when to go directly to the OR if there's an OR ready and so that would be one of those situations we can talk about that so I want to first of all round of a plus to our panelists. I wish we could spend the whole next 30 minutes just with questions and comments about this case but in order to get through this next amazing additional example of teamwork we will transition to the next case and then if there's at the end time for questions for both groups we'll do that. Thank you. While the transition I want to emphasize again what things Ben said these are some modesty going on here this went incredibly well because of what they did and so many of you did he mentioned that there was a life threatening bleeding emergency in the cardiac cath lab he didn't mention there was another life threatening bleeding emergency going in the app room at the exact same time I believe in neurosurgery and it was Friday afternoon and some of these people came in from the hospital. From the parking lot on the way out of the building so. Displace I believe is one of you place on earth that could have this child alive and you should be proud. Well thank you Dr. question. This next case or better yet series of cases is an example of how we saw how in the acute setting we can get together as a team and really develop a great outcome for a child this this next case series is a scenario of how we can identify a problem and come up with a very innovative solution with the resources that we have. The treat not just one patient but a series of patients in multiple conditions so I'm going to invite my colleagues Dr Schneider Dr. Baird Dr. Teginia Dr. Balkan. Leanne Jan Naomi if you're here and Emily to join me up here and we will get started with this next very interesting collaboration as well. Okay we have nothing to disclose for this or for the previous case. The objectives of this short discussion is to illustrate how this collaboration led to the it I'm definition essentially of a new disease entity how we are going to measure it. We can see the difference between the three patients and the three patients and be able to screen for patients that would meet these criteria and also how we treat it surgically. So this all starts with a 17 year old female who came to us with a short and subreth and difficulty swallowing right you can see from the CT scan how there's pretty much no space of the thoracic. So this little space for the trachea that's fairly compressed and the esophagus totally displaced to the side it's very rare when you see the esophagus bigger than the trachea in this location you can see here in the side views that just like the nominator artery is totally compressing the trachea. It is not just the nominator artery it's the space so think about that. So we have some of some of our patients that have had these condition very compressed trachea difficulty breathing recurrent infections and also difficulty swallowing from compression from essentially what's compressing are the great vessels or the spine or the or the aboney structures. Now so we have this challenge right we have a narrow thoracic inlet just not enough space to breathe the rate how can we make more space we can remove structures but ultimately what we need a space we need to expand the thoracic element and that's the key concept here today. We sought to assemble a team of experts and resources that we would need to accomplish this and this is just a few of those experts that have been involved in this process as many more that don't kind of fit into this slide but anything from cardiac surgery cardiology pulmonology nutrition genetics, your nose and throat anesthesia, plastic radiology orthopedics you name it because there are so many structures in the thoracic element. Dr. Snyder is going to tell us a little bit about more of this disease process. So the idea was thinking about the tracheobronky on the soft joe obstruction is basically provoked by the vascular compression either by the aortic arch or the enominate and it's induced by this narrow thoracic inlet which is formed by which is formed basically by the sternum spine in the first rib. So here Ben had demonstrated normal versus compression of the trachea by the aortic arch above the sternal notch and here we see the enomin arteries compressing the carina. So this is different from an entity that we deal with in orthopedics with congenital deformities which is called thoracic insufficiency syndrome where it's basically the spine in the rib cage that's unable to support normal respiration. And that basically causes an extrinsic restrictive lung disease. This is much more proximal. So what we did is we identified 12 patients that actually had these symptomatic thoracic inlet insufficiency which had a range of diagnoses and the presenting symptoms though are respiratory difficulty, coughing episodes, exercise intolerance, recurrent respiratory infections and dysphagia. The associated musculoskeletal deformities often are pectosex, gavottum, thoracic hypokiposis, scoliosis and then basically itrogenic causes such as a previous thoracotomy. So in thinking about this the idea was can we come up with a radiographic metric for the thoracic inlet to look and prove its sufficient or insufficient. And we had to deduce normal versus the diminished depth of the thoracic inlet formed by the bony margins of the sternum, vertebral body and first ribs. So basically we stole the idea of the halor index for pectosex, gavottum and came up with this thoracic inlet index which is the ratio of the thoracic width between the first ribs divided by the at the enter poster depth of the sternum to the vertebral body. And by doing this index we basically normalize for patient size and age. We evaluated 40 normal patients who had thoracic CT for other non-ISK diagnostics and we compared the index to patients who were clinically exhibiting the symptoms of thoracic inlet insufficiency. We did a receiver operator curve and basically were able to show that it threshold greater than 2.6 was relatively sensitive and quite specific and then we showed that it was age and size independent. In terms of thinking about the surgery as many of you know I'm an engineer so the idea was to think about this as thorax is basically this elliptically shaped tube hinge posterially at the spine. And if you open up the tube you're going to basically increase the perimeter and then increase its diameter and then this is a little bit of trigonometry to figure out how to calculate how big when you split that sternum you want to interpose a graph to maintain the atap diameter that you need. So my name is Emily Icoff I'm a biomedical engineer and I'm part of the cardio engineering team that operates in the heart center and my team got got pulled into this case due to the cardiovascular involvement and as you can see so we'll start with a patient's imaging so in this case CT and create these types of things. So this is digital multi component 3D models of the existing anatomy so as you can see on the screen this is a video here of the bony structure so the sternum the spine the aorta p a's the airway esophagus all these structures could be visualized and importantly these 3D models create a substrate a common substrate for all these different teams to look at the patients existing anatomy. And then we were able to take this a step further and actually perform virtual surgery and put in you know what would it actually look like in this patient if we put in an expansion piece of this dimension so we are able to simulate actually performing the midline sternotomy and distracting the split sternum open rotating about the spine. And then as you can see on the right here we were able to create a full size 3D print of this patient's kind of target post op anatomy which was used ahead of the OR time to pre contour the support stress that were used in the OR. So these are just some examples of the type of vascular reconstructions many of these patients are most all of them have some effect from either the aorta with the break you see the phallic vessels that compress on the airway and esophagus so we have to come up with some time some unique ways to rearrange these vessels in order to relieve that compression so the image on the left there is just a patient that we had to actually elongate the aorta because it was compressing. On the airway in such a way that it didn't allow the patient to breathe normally and then the image on the right there is where we had to move the enominate artery as it came off more posterior and had severe compression on the airway. And then in terms of surgical technique with regards to the trachea there are some techniques we've come up with and these are some images to kind of show that the image on the left we can go through that one first you see the esophagus has been rotated leftward and then the airway is then you know we're back up and we said that. And then you'll see we'll start with the left the image on the left will have the esophagus that is mobilized and rotated rightward and then you'll see that there's some sutures placed on the airway is what we've defined as. I'll start the work I guess I'll go back. A posterior trachea pexy. I don't know if that's not working it feels like every time I move it somebody else moving it I don't know if you guys are moving it back there. It's forwarded. And going backwards and going forward. Let's see if that works now. Alright so you'll see we'll move faster the esophagus is coming rightward the trachea is going to be pexy back. And at the same time on the right of the screen you'll see the anterior trachea pexy which was an operation we define because we realized the denominator of a pexy didn't work great you'll see the is airway now being supported anteriorly by the direct sutures and back to the left you'll see the airway being supported post yearly. With the sutures and that gives us nice now support of the airway now therein lies the problem if we just pull the sternum back together we recreate the same problem and therefore we need to elevate the sternum anteriorly these are just some examples of pre and post looking at the airway after expansion and trachea pexy. Okay. So the idea was how do you then reconstruct the chest wall so originally we came up with this idea of maintaining this gap between the split sternum so the first several patients I harvested rib and then created these little bridges and sew them in place more recently. And a lot faster is I use a iliac crest aligraf shape it to the pre planning. Shape and dimensions basically using an iliac aligraf and then I help support it with a titanium plate just in case we have sufficient stability. Perhaps the simplest part of all of this is what we do in plastic surgery to assist we think it's very important to cover this bone aligraft with well vascularized tissues reasons being number one we think it helps you know support. Vascular ingroth we think it helps deliver antibiotics and we think it's important from a secondary standpoint that should we have when killing difficulties exposed muscle fears far better than exposed aligraft and so you know the workhorse that we utilize for this another chest wall reconstructions is really the pectoralis major muscles. You know based on the pectoral brands of the Thracrachromial vessel you know we mobilize these muscles as much as we need to offer the sternum off of the clavicle off of the the caster cartilages and sometimes off the external obliques and only when necessary do we really take its origin off of the humorous and so our goal is to really advance these muscles and bring them towards the midline when necessary we need some supporting roles and so when necessary above we can. Not harvest we can mobilize the external cladal mastoid muscles and below we can use directus muscles and our goal really is again to provide well vascularized muscular coverage over this bone aligraft. So I'm going to go me from cardiac surgery so this slide is just looking at both the pre and post operative symptoms and going through them but we initially saw overall respiratory function improved in all patients that underwent a sternal clasty. At late follow up 10 out of 11 patients improved respiratory symptoms from a pre and post operative baseline and then eight of 11 patients had full resolution of respiratory symptoms so breaking them down we looked at both reoccurrent and severe or severe respiratory infections chronic or creepy cough disnea oxygen requirements cyanosis or solid food dysphagia in regards to the solid food dysphagia there was one patient that presented and they had four. So this slide just goes over that we are able to obtain both pre and post operative imaging for six out of the 12 patients and it demonstrated that there was an increase in the anterior to post amener diameter depth by 19.6%. So looking at the images to the slide you're getting at a starting diameter of 22 millimeters of thoracic inlet and it was expand to 35 millimeters which is giving you an overall increase of about 59%. Looking at the all these patients got both pre and post operative bronchoscopies and the pre operative bronchoscopy all of them demonstrated that there was tracheal compression at the post operative bronchoscopy that there was relief of this compression to fully actually quantify this we actually looked at the CT scans and measure them and they all were all demonstrated an 87% increase and their AP tracheal diameter. So looking at the slide the initial tracheal diameter is 4.9 millimeters or six using a 7.49 millimeters and the finishing tracheal diameter is 12.7 millimeters and this correlates to a 70% increase in diameter. So what has this experience done for us? It's really done incredible things. This is an example of a patient that presented to us from an outside hospital in extremists from birth and presented with an LV infarct and it's amazing the child survived initially. So then we had to take this child to the operating room and do what we call a stage one norwood operation on the child and the time post operatively required to recover was the long time in a matter of weeks post operatively and what happened is the sternum basically retracted on itself. And so there was a large gap in the center of this child's chest that we just couldn't bring together we tried and it would compress the heart. So at the time I reached out to our colleagues here and said we need to come with solution as the child now is slowly recovered and what we came up with was along the same idea. And so what was done is a piece of bone from a legraph was taken cut out and we interposed it in the sternum of this child and amazingly this child recovered and essentially was discharged and more amazingly what we learned he came back 19 months later thriving alive in his ready for a second stage operation. And probably the most amazing part to us was the bone had actually had neovascularization and we didn't know that that would happen I think there was some assumption it would happen and then so we reoperated on the child and then we're able to then take another piece of aligraf added to that aligraf and close the child in the sense been just charged so really it was one thing just led to another and they continue to do that. And a number of aspects with this experience. This is just another example of that that same case and that that Dr. Bear just mentioned you can see in the middle there's that aligraf that's interposed between the pre existing aligraf that was bleeding and it was bleeding quite extensively which we were all quite happy to see. And that again these these pectoralis major muscles that were previously advanced needed to be advanced much more and so that they were harvested and bilaterally and lifted internally and brought over to cover the the sternum aligraf. So in summary of these two cases showcase the benefits of a multidisciplinary surgical collaboration with rare in atypical pathology it really pushes us enforces to think outside the box and to do what I like the term cross pollination of ideas we all share our different ideas and I hope that with you seeing these examples you can think of other scenarios where this can be helpful or these type of collaborations can be fruitful. As you saw we defined new disease entity insert solution now we're getting doctors getting phone calls from all over the world with patients just like this that made benefit from this and we you saw how we use this to extrapolate this technique to other disease entities as such as the one Dr. Bear just talked about. We had amazing resources of our institution to maybe be able to accomplish this the virtual sort of planning you saw the collaborative environment the or infrastructure and nursing was essential for this and the team approach was was again one more of a highlight so I want to thank you for attention we have big 10 minutes for questions so feel free to the questions and comments and we appreciate that thanks. First simplicity I apologize to the people on zoom we have to take questions from people in the room I do want people on zoom to know that the folk military is entirely full a little bit just ran out for the code and for the people in the room to know that there are 230 people on zoom so I hope this has been worth everybody's time and these guys have had a high bar and I expect will be hearing about lots of suggestions for more cases so we'll take some questions and we'll see you guys in the next video. Can you do a room and take them on this case? I think you or but yeah you can start with this. I just wanted to ask Emily I hate to put you on the spot but you know it's it's pretty unusual to have a you know card carrying engineer very accomplished engineer addressing these kinds of medical problems at the bedside. Can you comment on what what you do and what you know what you feel is a contribution that you're bringing to this. To the care of these kids. Yeah thank you for the question I think I think as I said like being able to create a. You know two scale model of the existing anatomy. Where everyone is looking at the same thing no one has to reconstruct in their mind and have you know because. Right now if you're just looking at a CT scan kind of scrolling through it you have to recreate in your mind like what that might look like and my model my mental model might looks different from somebody else's mental model and so when you actually have. These digital 3D models of the existing anatomy it really just kind of levels the playing field everyone's looking at the same thing and then it allows people from all these different teams who are caring about all these different systems and and parts within the case. So I think it's really important to have this to talk about the same patient and it becomes very obvious as well when you're looking at these models like oh I can see this is where the airway is compressed and that's because you know the aorta like this part of the enominant is going over the airway in this part and I can see exactly like the sternum and the spine dimension is very narrow here I can see exactly why this is being compressed. I really clarify is in some cases what is wrong and what needs to happen to correct it. Hi this is Scott snapper from GI I actually don't have a question I wish I did I really just have a comment I you know these cases really just remind me and I'm sure reminds everybody else in this room how unbelievably special it is to be at children's home. It's it's and I find this is the first time I've been in a conference like this the best of the best and it's a paradigm for others and I just I'm just stunned. I think this does highlight the the special sauce with Boston children says everybody that you heard from is an expert in their own right and they work in their own lane but when you have something complicated it's really nice you can go down the hall and find something and every other is this lane who wants to help yourself a problem that's what makes this place so special we have some more time for more questions. This is a perfect example of innovation at Boston children's trying to solve a difficult problem so the team had identified a clinical problem with no solution and solved it so to me this is a perfect way to ideate about rare diseases I mean that's why we're on the competitive frontier right we do things that nobody is able to solve maybe as chairman of departments we identify specific diseases that don't have viable solutions and put a crack team like this to be able to solve it I mean this open stuff. This opens up a whole box of solutions for rare diseases so I don't know how you'd operationalize something like this but I think that's something I was thinking as Ben was presenting this new disease or at least a solution for a disease that wasn't solvable before. I think that's really the whole point of doing these super balls is that these people have self organized they don't have to go to their chiefs to say who could I talk to about this or that some of them may not have known each other before but they talked to one and then they said I've got this colleague who can do this but there are others who it's not in the in our general nature to go ask for help or to say I don't know what to do so the whole idea of this is to do this. I just want to create this new you where everybody says well I don't know what to do with this and my partner doesn't know what to do with this let's go down the hall let's go across the street let's go to the scientists this this is what we want to stimulate so I'm hoping that I'm going to get a lot more suggestions about doesn't have to be five services for the case but cases that we can do a couple times a year to let each other know how to stimulate and who's so either I guarantee you there are people up here that some of you never heard of. I never met just because we're really big place. Time for one or two more questions. So first compliments is these are amazing cases and a great idea. I was fascinated on the pre-imposed up CT how not just the width of the chest but the distance between the sternum and the spine probably look like it may be tripled. So what is the mechanism for that why does that get so much bigger. So basically just think about it so the chest is is basically it's a tube so if you increase the diameter of a tube by definition you have to increase the A to P diameter so I skipped over the math one that but basically you can calculate exactly what you need to do. So basically if you go like this then I'm increasing the circumference by definition you've increased the cross sectional area and in this case it's basically you calculate from our we calculated a thoracic index for normal of 2.6 you see what the patient is you basically then say what the new distance has to be and it's basically it's a right angle triangle and you just saw it's the arc 10. I won't go into the math but anyway you can you can calculate it and then we put it into our pre-out planning and then we're able to say okay I need a shape of the sternal I'll go to the aloe ver to be X. Thanks this is a wonderful case I think great example of teamwork and innovation. I've been just asking a quick question about the first case and that is we do not do a lot of direct to the O.R. admissions and that is somewhat distinct from adult trauma centers or places where they manage for ectaphoresis for pharmacoprolytic aneurysms or thoracic aneurysms. Did we learn from this at all about some of the things we might do differently or could you smooth around I was sort of peripherally involved in that case and I agree with you the work was outstanding the collaboration was fantastic there was a little confusion about whether this patient was going to come direct to the O.R. or not. not something we do a lot of. And I'm just wondering if we took any lessons out of this about that. I think this would have been a good example for that. I think that the challenge was we didn't have a lot of time for notification. So I think in another scenario, we had a little more time and we had a or available that certainly could be the discussion that this is a child who has to, we know has a battery that has to come out. So one of our target metrics that need to come out within, you know, as soon as possible, right? And so it's not like whether he needs to go to the OR or not. He has another reason why he needs to go to the OR, he's believing in the death. So this would have been a good scenario of like, okay, do we have an OR groom set up for resuscitation? And yeah, when I was John C. Risen, we had aortic aneurysms from the helicopter pad down to the OR because we were ready for them kind of thing. And so I don't see why this wouldn't be a set up so that if we could establish some education, some pathways to do something like that. Because I mean, we were super quick in the ED. This was one of the quickest sort of in and out so the ERs I've seen in a while. So it's less than 20 minutes. In the 20 minutes can be big difference for some kids. It wasn't for him because I think that he had sort of auto-tomp and outed with that amount of clot in his esophagus. But if he hadn't, it may have been the difference. Great, we're right at time for those that don't know that clock is three minutes ahead so people don't go late. Let's actually eight o'clock right now. I want to thank everybody. I want to thank all the presenters. I particularly want to thank Ben for organizing this. And please let me know when you have suggestions because we're going to do this twice a year. If people like it, if it's not worthwhile, let me know that as well. Thank you everybody. Great job. I'm going to have to ask you for questions. Yeah. I heard a video about this topic.