Dr. Mark Proctor and Dr. Susan Goobie - Pediatric Craniofacial Surgery: How to Improve Care

From the Department of Neurosurgery and then Doctor Susan Gooby who's from our anesthesia department. Um, so I'll just say a little bit about both of them here. So Doctor Proctor is a professor of neurosurgery at HMS and has a clinical focus in craniofacial abnormalities, spinal disorders, and trauma of the pediatric brain and spine. Uh, he completed his pediatric neurosurgery fellowship here in 1998 and since then has worked to build the neurosurgical spine and craniofacial sections within the neurosurgery department. Um, he's chief of neurosurgery and director of the craniofacial and spine surgery and director of craniofacial and spine surgery at Children's Hospital. Um, he's recognized nationally and internationally for his contribution of several novel approaches for craniofacial and spine spine surgery. He was also instrumental in the multidisciplinary development of a pediatric pediatric interoper brain, pediatric inoperative MRI imaging. And was the neurosurgeon on the team that brought this technology to children. And Doctor Guy is an associate of, uh, anesthesia, associate professor of anesthesia at Harvard Medical School with an interest in craniofacial scoliosis and neurosurgery. Um, she has expert knowledge in blood conservation methods, coagulation disorders, and pharmacokinetic research. Her clinical expertise and research is innovative as it provides clinical guidelines into optimizing blood management in pediatrics and particular uh and in particular on the antifibrinolinic use for pediatric non-cardiac surgery. Uh, she's section editor for the pediatric anesthesia and a regular reviewer for major anesthesia journals. She's director of the BCH Pharmacokinetics lab. And she is chair of the International Pediatric Craniofacial Surgery Perioperative Group and the chair of the Society for Advancement of Blood Management Research Committee. Uh, she is an internationally recognized expert and investigator on several large multi-center trials in the area of pediatric blood management. Please join me in welcoming Doctor Proctor and Doctor Doctor Guy. Great. Thanks for the invitation to speak. So what Susan and I thought we would do is sort of divide the talk into two halves because we've, we've sort of approached safety and efficacy in craniofacial surgery in two ways. And one is by altering the surgery we do, and the other is the sort of the patient selection and anesthesia technique. So I'm going to cover sort of the basics of the condition, what is cranial synostosis. And how we manage it, the two different, very different types of surgeries, and then Susan's going to talk about all the things that we've done and where she's really been a leader in changing the anesthetic management. Uh, these are just the routine disclosures. I have none, and Dr. Gubi has a couple of, uh, ones that are not pertinent to this talk. So, again, I'm really going to focus here on discussing the diagnosis and surgical treatment options for cranial synostosis, and these have evolved quite a bit over time, and we've been sort of one of the leaders in moving to the more minimally invasive techniques. And Susan will then present and she'll go over this again, uh, looking at how anesthetic techniques have changed and how we have collaborated with other centers to really see what works and what doesn't work. So I'm gonna start really basic here about, you know, why, why is cranial synostosis even a, even a condition and start with the fact that our skull is made up of 5 major bones. Uh, so there's the 2 frontal bones, the 2 parietal bones, and the occipital bone, and you could say, well, first, why does this. Why is this even necessary? Because for most of the bones in our body, we don't think of them as, you know, the arm as being made of several separate bones, although early on it is. But the skull we really do, and we move the bones and we do all sorts of things to the bones. And it's because it, it, you have to go back a little bit in evolution to when we went from walking on four legs to walking on two legs, right? So when that happened, the pelvic inlet really changed substantially, and you can have something like a little baby calf be born, get up and walk away from its, from its mom because its brain is advanced enough to do that, whereas baby humans can't do that because their brain is born in a much earlier phase of, of its development. And that's why it takes about a year before they can start to walk and talk and do those other things, because that first year of postnatal life is essential for brain growth. So if we look at that just on a growth curve, you can see this is birth, this is 3 years. By, by about 6 months, you've achieved half the brain growth you're going to have in your entire life, and your brain is actually about 80% of its adult size. So you look at this little 20 pound baby that's going to grow maybe 10 times in weight. The brain is 80% of where it's going to be. So brain growth happens very early, and you can think of the brain almost like air filling a balloon, right? So if you think of the skull as a balloon, as the brain is growing, it's pushing all of these bones apart. So if, let's say you have this bone here, as the brain it's growing, it's pushing these two bones laterally, and you grow in width. And if those bones are closed, this is Virkow's law. If those bones are closed, you start to grow in length. So Verkow's law is very simple. It just states that skull growth is restricted in the plane perpendicular to a prematurely fused suture and enhanced in a plane parallel to it. So if you think about that again, if this is closed, you can't grow in width. That's perpendicular, so you grow in length where all the other sutures are open. So that's sort of the basics of what the condition is and how common is it? Well, we see it about 1 in every 2000 births. So about 1 in every 2000 children being born is going to have it. So you may never meet a baby that's had it because that's relatively rare. Uh, sagittal is about half of it. So 1 in about every 3500 have that suture that I just talked about being prematurely fused. And then all the other forms become much, much more rare, and then you get into the dromic conditions. And we see a lot of APR children here just because we're sort of a national specialty center for it, and that's about 1 in every 30,000 kids. So we'll see maybe 10 AR children a year, but that's a very high volume for that condition. So now if we sort of just look at what are the different things that can happen if you go from that normal skull, this is just to sort of give you a general sense of the skull shapes we see and that may roll, roll on that gurney down to the operating room. So this is saggy synostosis where this bone is fused and you get that long narrow skull. This is metopic where this suture up here is fused and you get a very triangular pointy skull. This is bilateral coronal where both of these are fused. So you get a short wide head in the front. This is, if only one of them fuses, it's unilateral coronal and you get just flat on one side and then this is if a bone in the back fuses. And then far, far more common. So if this, if all of these are about 1 in every 2000, this is about 15% of all babies when they walk into our office or are carried into our office, we know that the head is misshapen simply because they've been lying on it and they have soft baby heads and with the back to sleep program they're lying on the back. So if they lie over here they're going to flatten that and push up the front of the head. So, so again, this is hundreds of times more common than all of these others combined. So now we'll just sort of go through some of the, some of the basic ones we see. So this is a case of saggy synostosis. So positioned here for the operating room. The nose is up here, the eyes are here, ears, and it's a very long, narrow head. So normally the back of the head should always be the widest part of the head. So this part should be the widest, and you can see it has this what people call a bullet shape or scapylocephaly, meaning shaped like the keel of a boat. So that's how it was. Originally described as scaphylocephaly, boat-shaped. This is unilateral coronal. So this is when this suture is open here, this one is closed, and as a result, you get very flattened on that side. The nose tilts towards that side and the eyes are uneven. And here's something called the Harlequin eye sign, which you can see, which, you know, you may, may not have heard of. That's sort of the classic radiographic finding for unilateral coronal synostosis. This is metopic synostosis where these two frontal bones are fused and you get, it's very pointy and you get that ridge coming down the middle that you can see here on the, on the CAT scan. And this is just metopic after treatment. So this is a child for whom we did a little operation, opened the bone here, uh, or opened the skin here, removed the bone, and use the helmet. So you can see those changes over time. And we're going to sort of walk through the two different types, the open and endoscopic treatment. And then this is lambdoid synostosis. So this is when the bone in the back here is fused, and this is very rare. So we see here at this hospital where we're a very busy center, we see about one case a year of true lambdoid synostosis in an isolated fashion. So now I just, I want to go through a little bit, you know, now we've defined what the condition is, how do we, how do we treat it? So for, for years, strip craniectomy was the way that this was treated. So you would just open the skin and you would take out that fused bone and let the brain growth take over. It, it hit a couple of snags. One is that it was being done a lot on kids who had what's called secondary cranial synostosis. So they had just very abnormal brains, right? So if early on I said you need growth of that brain to push the bones apart, well, if the brain is not growing, the bones don't push apart and they fuse. And opening the bones doesn't do anything because you don't have an underlying brain to push it apart. So these strip craniectomies were first done in New York in around the turn of the 1800s to 1900s, and a lot of kids were dying from surgery, so they were being done for the wrong indications and it wasn't very safe, and that put a complete squash on craniofacial surgery for about the next 50 years. No more synostosis surgery was done for about 50 years, even though there were real cases. And then the problem when they started doing it, and Dr. Shillito here was a big proponent of strip craniectomy, was about a third of the kids fused the bone back too soon. So you, you open up that fuse suture, but it fused back before there was any appreciable change in the head shape, so it was considered sort of a failed surgery. Uh, so then we went to these bigger techniques, these calvaal vault remodeling operations, where you don't just open the bones, you take them off and you move them into the right position, and that was very successful, and we'll go through caser, but as, as Dr. Guy will tell you, it's a very big operation for very small children. So there's a lot of anesthetic problems. There's big transfusion requirements and they're long 6 to 8 hour operations. So, so these are were considered, you know, effective operations, but not the best thing for the child. And then we got some uh new technologies that led us back to strip craniectomy. And what that was is the combination of the helmeting, uh, so that after surgery we can use a helmet to reshape the head. And um the fact that we're now able to do these in a very minimally invasive way so that we don't have to do these big incisions. We could do really small incisions and go from there. So that we've gone essentially from these open procedures, which are a mechanical operation, you know, you remove the bones, you move them around, it's sort of like doing Legos to an endoscopic surgery where it's a release procedure and you're relying on the growth of the brain to move the bones and you're using something like a helmet to direct that growth. Now, interestingly, it hasn't caught on as much as you would expect, right? So we're a fairly large center in this. On Monday, we'll do our 496th case, which is the, probably the second busy, uh, busiest or biggest experience in the, in the world. Uh, so we're, I know that because we're getting very close to 500, so we count each one. But it hasn't been, uh, it hasn't been adopted widely. So for this audience, I wanted to sort of compare it to laparoscopic cholecystectomy here because that didn't, that didn't sort of jump to the front of the, the line, uh, when it was introduced either. So it was first reported in 1987 and it met significant early skepticism. And how do I know that? Because I went back through the literature and You could see some of the, uh, some of the original commentaries on lap-Cli, la laparoscopic cholecystectomy, passing fancy or legitimate treatment option. Laparoscopic cholecystectomy. Let us control the virus. I'm sure these are sort of like, you know, what you guys are up reading every, every night, right? Laparoscopic cholecystectomy, fantastic question mark. You know, that's, you can, you can just sense the sarcasm in that statement. Uh, and then finally, you start to see laparoscopic cholecystectomy evolution, not revolution. People are starting to see, hey, maybe this is a valid way of treating this condition. And then Then some real evolution where, you know, they sort of accept it as a treatment paradigm, but say only surgeons involved with open cholecystectomy and management of its potential complications should perform the procedure, right? And now I would sort of assume that most of us, if we wanted our gallbladder out are going with a lap coli, we're not, we're not asking for a big open operation. And this, this is sort of we're, we're in that evolution now for craniofacial surgery. So, there's a lot of centers that still will not do it. They don't believe in it. They're not comfortable with it. Uh, and we, we had this, uh, Consensus statement, not a consensus statement, the parameters of care that came out several years ago. So I was the lead neurosurgeon on this. It was a big, big grant from the CDC, and, you know, basically it said, well, endoscopic surgery is actually a very legitimate treatment option for this condition, but it should only be done in centers that know how to do the open operations that can really care holistically for the patient. So I'd say it's sort of heading very similarly to LabCli but isn't quite there yet. So now I just wanna go through, you know, give you guys a sense of what we do in the operating room, both in the open technique and with the endoscopic technique, and then Susan will take over and talk about the anesthetic management. So I wanna show you here sort of a typical open repair for saggyl synostosis. So you can see that long narrow head. And this is the incision. So these are all done jointly with John Mira, by the way, and John, uh, we asked if he can come help with this talk, but he's not here today. Uh, but I, I don't want to make it seem like the team is just Susan and I. It's very much a joint team with plastic surgery. So we make these zigzag skin incisions and that's because they hide better under the hair. So if you make a straight incision, the hair splits around it, but that zigzag gets covered. And here you can see that long, narrow head, especially long in the back. And then this is, uh, you know, John, uh, came here from Melbourne, and this is a technique he created called the Melbourne technique. But I just want you to look at the complexity of this. Like, you literally have to label each bone or else you will forget which one was which on the back table. So he uses a, a marker that, you know, A, B, C, D, you know, etc. all the way up to F, um. Here you can see a child before, before surgery, that long narrow head. Here it is at surgery, literally labeled, right? F, A, C, V. You know, you have to, you have to remember which, which one was which. Then I get the uh the fun of cutting on the dotted lines, right? So I go through and I take off all those bones and here we are with all the different pieces again that are labeled so you know where to put them back. And then he, you know, then there's another template for where to put them back. And uh again, it's like it's sort of like Legos. You just have the instructions, you follow them and you go, you go back and put it there. And that's what it looks like at the end. Uh, so saying like this piece of bone here was here, but there's a piece of bone in the back that used to be here that's now lifting up the back of the head, you know, it's fairly complex. And I want to contrast that with endoscopic surgery. So this is a setup for endoscopic surgery. So we have this sort of special head holder, baby facing forward, tube coming out here in the front. Uh, these are the outlined incisions for that operation. Uh, this is just sort of getting ready for it. This is just a standard endoscope. Frankly, we often use a urology scope because it's not very fancy endoscopy and we don't want to hurt the scopes we use to work inside the brain. And I'm just going to take you through a 2 minute video that shows the operation here. So this is setting up the specialized head holder. Here we have the head where we're infusing two incisions, one in the front, one in the back. We just make small incisions as you reach about 2 centimeters. We use the uh Colorado needle to come through it. And we're coming down to the periosteum. There'll be a really palpable ridge under each one of those where the, where the area is, and here we're drilling right through that ridge. So coming down, enlarging the little hole that we've made there. So we use things like kerosenes and other bone, bone rejours and then once we've done it in one, we'll go do the same thing on the other side here. This is actual speed. The operation takes 2 minutes. And here we are enlarging it and in truth, the operation takes only about 25 to 30 minutes. So it's uh, it's not obviously not full speed, but it's a pretty quick procedure. And now here we're starting to work where we're getting ready to move in the endoscope to do the next phase of the operation. So here you can see through the endoscope, lifting up one of those skin incisions. Here's the dura over here, and here's the bone. And our next goal is to separate the dura from the bone. And here's the deep ridge on the inside from the uh Sagyl synostosis. And we'll just sort of go back and forth a few times to be sure it's all free. If there were any vessels uh bridging there, uh, here we've got up to the front hole. Sorry, I missed it. If there's any vessels, uh, we just use a bipolar to take down the connections. And once we know that that's all free, we'll take out the scope and use some really crazy looking scissors. There it is. So that was the other hole. Uh, so these are called Tess A bone cutting scissors and we just Connect the two, the two openings there. So, um, It's done from one side with the scissor, then from the other side, and then we'll pull out the strip of bone. And in this case, it comes out into, two separate pieces. Uh, sometimes it comes out in one. So that's the bone coming from one side and there's the cut in the bone coming from the other side. So, as you can see, it's a, it's, you know, as operations go, these are very vastly different types of procedures to treat the same. The same condition. Line it with gel foam and we just close the incisions with dissolving sutures and, you know, that's, that's really it. And this is just showing taking out the bone. This is a case where the bone came out in one piece, so we adequately connected it. Here's the incisions, and then this is the baby in a helmet, and this actually starts within 5 days of the operation. A baby goes in a helmet, which is now going to restrict the front to back growth and leave lateral growth. And again, that helmet just sort of works as a shield and in this case like we need growth here, it's going to force the growth in here. Some centers are using springs, some distractors, those work as well, but they do require a second operation to remove them. So if we're trying to make this minimally invasive and less surgery for the child, adding that second operation isn't necessarily the best thing in our view. And these are helmets for other conditions. This would be from a topic where that pointy head in the front and that's sagit. This is bilateral coronal. And here's just to show you some of the changes you get over time, right? So if this is how it starts again, front of the head, back of the head, that prominent back, this is it at the end of treatment. So it's very effective. It just takes longer. You don't get an immediate result. It takes about 6 months to get that result. And here's just another, another case looking at that. And the final thing, just because we're in a big era of value and our accountable care organization is, I just want to say, well, is one way more economical than the other? And John Muir, I'm quite sure he did this study because he thought my way of treating it was more expensive, that the helmets, etc. were going to be a more expensive way of treating it, and he wasn't a big fan of it because he had, he had a super duper Melbourne technique with 14 different pieces of bone. So he did a big cost study and lo and behold, the big operation. And this is all in one year costs including gas money to get to the, to get for your helmet fitting, etc. The cost for the hospital stay, the surgeons, the anesthesiologists. The big operation, the CVR cranial vault reconstruction, was $55,000 and the endoscopic surgery $23,000. So from a value perspective, we're looking at an operation that was 40% of the cost and three other centers have now shown that same, that same thing. So if we're looking, you know, for benefit of the patient, much smaller operation. Much cheaper and we're, you know, we're not going over the results here, but I can assure you the results are, are uh really quite favorable. So that sort of finishes the, the surgical side and now uh Susan Guby is going to come up and talk about the uh the anesthetic components of it. Thanks, Mark. Uh, thanks everybody for coming and thank you for inviting me to speak today. Uh, so I'll continue along and take another 2023 minutes to, to finish the talk and then we'll take questions. Um, so certainly craniofacial surgery and craniosynostosis surgery is really one of the most challenging pediatric surgeries for the anesthesiologist, uh, because there's usually rapid massive blood loss, uh, in a small patient. And listed are some of the complications and considerations that make these patients really very, very challenging to manage rapid blood loss in a small patient, uh, comorbidities, difficult airways, long operating times sometimes, and patients with increased intracranial pressure, and you can see some of the pictures of some of the challenging patients we've, uh, we've taken care of. And although surgical and anesthesia techniques have really been refined over the years, craniosynostosis surgery is still to this day associated with substantial bleeding. I just went to a meeting of anesthesiologists who do craniofacial surgery all over the United States, and a good number of them are hanging blood on incision because the blood volume losses varies between 1 and 2 blood volumes for these, for these babies. So I propose that multimodal bleeding management really can improve care, and a lot of my talk will focus on the, on the bleeding management of these babies. Complications include these listed, and mostly these are all directly related to blood loss and the consequences of massive transfusion. So in an effort to improve care, I wondered which patients are at higher risk and which patients, you know, have these complications. So we undertook a large retrospective study and it was over 10 years from our Boston Children's Hospital database. All of this was manual chart review back at the time. It was from 2003 to 2013 and reported this in 2015 in anesthesiology. The incidence of a major postoperative adverse event that required an ICU stay in our patient population at that time was 13% overall, with 15% of those being cardiorespiratory and 30% major hematological. Uh, conditions. And so we thought we'd look at what those risk factors are. So we did multivariate regression analysis and looked at all the independently associated So this isn't cause and effect, it's those variables that are highly associated with increased adverse events in our craniosynostosis population at Boston Children's Hospital, and these were the significant predictors. Body weight, less than 10 kg. If they got a massive transfusion, red blood cell transfusion greater. And 16 mL per kilo if they received any hemostatic products, which are FFP platelets or cryo, if they did not receive antifibrinolytics, if they were ASA 3 or 4, so very complicated patients, usually syndromic, or if they had an intraoperative complication, all of these were independently associated with higher risk. Um, and then we wanted to sort of plot it out, and another way to look at it is this figure, and it shows the percentage of major adverse events for each category, and this is a little hard to read, but these are all of the six different independent risk factors that we had determined in the previous slide. And I'll just point your attention to these two. Clearly the largest differences were for red blood massive transfusion or if the patient received any plasma hemostatic products. Um, and the differences were increased by 65% and 47% respectively. So clearly bleeding and massive transfusion is not, is not only associated with adverse events, but really clinically significant morbidity and mortality in our patient population. So given that the main hurdles hurdle is bleeding, and we didn't have any good guidelines for bleeding management in our pediatric patients. In the literature, I proposed these pediatric patient blood management guidelines, and they're basically three different tiers or pillars of intraoperative, post preoperative, and postoperative care. And so I'm just going to highlight a few of these modalities that we particularly use for our craniosynostosis patients. But before I do that, protocols are fine and guidelines are fine, and they work very well. Uh, but I just wanna talk about the miracle of hemostasis. You may remember the coagulation cascade with the intrinsic and extrinsic pathway. Well, this figure, um, sort of represents that and how it's a little bit of a mystery. There's one more really important factor that you might not have heard of. You've probably heard of factor 1 through 13, but what about factor 14? And here's a little hint, um, so, uh, factor 14 is, is something that anesthesiologists, uh, Deal with every day and it's our surgeon. Um, so I'm very fortunate enough to work with, uh, two amazing surgeons, uh, Mark and John, and they're very excellent at, at limiting the factor 14, uh, contribution to the, to the day. Uh, but I just want to highlight what um the very smart person said, Jim Denardo, in an editorial in anesthesia and analgesia. He said we must work closely with our surgical colleagues to stress the importance of meticulous hemostasis because there's really no evidence that all of our multimodal blood strategies, blood techniques can uh prevent large volume blood loss. So thank you, Mark and uh and John for, for helping with that. So as Mark Proctor's already told you, there's conservative surgical technique, which the main goal from my point of view is keeping the blood in the patient, and we've shown that those patients who are amenable to an endoscopic procedure compared to an open procedure, the red blood cell transfusion rate is a difference between 95% of our cases are open cases versus Uh, 4.6% of the endoscopic cases and that continues to decrease, um, um, with the, with the 500 that we've done, but 4.6% is the latest uh percentage of transfusion in those patients. Another really innovative conservative surgical technique for those patients who need the open procedure, these slides are from John Muirror, and it shows how we can plan or he, he can plan, the surgeons can plan with 3D models, especially for the very tricky cases. They use these models that are Generated by a 3D printer and um the surgeons, surgical residents, and fellows can look at these models preoperatively, plan their surgical approach, hopefully minimize the surgical approach and the, and the time so that uh I believe this is another way of limiting that factor 14. So I, I can't go on without talking about one more miracle of hemostasis, and that's uh. Factor 15, and that's us. What goes, what happens behind that, uh, blue drape and, and does it matter? And for the remainder of our talk, I'll talk about everything that happens, uh, behind the blood-brain barrier as we say. Uh, so, perioperative bleeding management guidelines, these are all the different strategies and that we implement, um, uh, careful blood pressure management, very careful fluid management to avoid hemodilution, but to maintain, uh, Uh, blood pressure, uh, using a restrictive transfusion strategy. There's very good guidelines to show that a restrictive transfusion strategy is safe. Uh, transfusion algorithms, I'll talk about critical bleeding protocol, antifibroanalytics, and cell saver. Um, and so what we did was we, uh, standardized the anesthetic technique, and I wrote a standard sort of guidelines or clinical pearls for our anesthesia technique which are on our website, so anybody who's doing these cases can pull this up. And they really go through all the pertinent factors of, of, uh, good patient care in our craniosynostosis patients, which include all of these different management guidelines. Um, one of the main, uh, in the, in the first blood management strategy that we started using uh in these patients was using tranexemic acid. We did a double-blind randomized trial and uh published it in 2011. And what we did is we randomized patients to either TXA or placebo for craniosynostosis surgery. And up until this point, it hadn't been proven to be effective in this patient population. Um, and when you look here at blood loss, uh, versus intra-op post-op and total blood loss, the red is TXA and the blue is placebo. There was a 55% reduction in blood loss. Uh, similarly, when you look at the total pack red blood cell transfu and the TXA versus the placebo group, there was a 60%. Reduction in red blood cell transfusion, and this was just by using TXA. There were no other protocols or anything at that time that we use many different anesthetic, uh, anesthesiologists doing this procedure. The only thing that was different was that we, uh, use TXA. We also reduced the FFP administration to 0%. Um, so that was one, blood management strategy that we, uh, implemented, and that's become a worldwidely accepted now for craniosynostosis patients to use antifibrinolytics. More recently, we've started to use intraoperative red cell salvage. Um, and we currently use CellSaver as a pilot project for patients greater than 10 kg. Uh, we started the protocol 6 months ago and we've had very much success in this older, uh, more weight, uh, higher weight group. We've had 6 patients and 3 of those, we've only given a Cellaver transfusion from the salvaged cells, and we've been able to, uh, not give banked blood, and 3, we gave a combination of both. So we're going to work on. More on this technique and this may be one other modality that we can use. So by keeping in mind management goals, we can improve care first and foremost, the goal of the anesthesiologist is to restore and maintain oxygen delivery to vital organs and tissues, to maintain normal anemia, avoid coagulopathy and anemia, uh, and treat with the appropriate products when indicated, avoid hemo dilution and overtransfusion at the same time. So we, uh, so I, I, I, um, proposed this bleeding management, uh, bleeding, critical bleeding protocol, uh, for craniosynostosis patients, and I took this to the transfusion committee and they liked it. Uh, the anesthesio uh, the anesthesia department has accepted it and hoping to maybe roll this, uh, Protocol out for uh Boston Children's Hospital worldwide. It's gonna go through the uh intensive care units and um the, the trauma committee, and this is part of it. Um, and I think we need something like this to help manage our, our, uh, patients, uh, in the heat of the moment. And first and foremost, we need to control the bleeding and treat the hypovolemia aggressively. And the protocol goes through, uh, you know, a, a list of different, in a, in a flow diagram, uh, point of, uh, point of care, um. It, it outlines all the uh goals for maintaining hemoglobin, fibrinogen, INR and platelets, when to transfuse and, and how and how to treat all the different uh sequelae of massive transfusion. Um, so back to our, uh, bleeding management guidelines, you know, what has it worked? Well, we were able to decrease transfusion from 95% of our open cases to, uh, 70% over the last 6 months. Uh, and that's still a work in progress, uh. Uh, but what about other modalities? Next, I wanted to look at preoperatively. How can we optimize um care preoperatively. It's not something we think about a lot. Patients come to the pre-op clinic maybe a couple of days before their procedure and then, uh, off they go to the operating room, the labs are sent and you see them the day of surgery. Well, this is a consideration which I think is very, very important. The World Health Organization states that anemia is a universal health problem. In industrialized countries, 25% of preschool age children are anemic. Does that pertain to us? It does. We looked at, um, uh, the incidence of anemia in our craniofacial surgery, uh, population. And we have the same as the World Health Organization reports that 25% of our patients coming for craniosynostosis are anemic. Um, and most of this in the older age group, the open procedures is iron deficiency anemia. We now have a pilot project that we're looking at over a six month period, screening for iron, rectic count, and TIBC. We're going to work with our hematologists to try to um And our uh craniofacial clinic and, and All the patients, all the people who take care of these patients preoperatively to try to optimize that. Why does that matter? Well, there's a 60% higher odds ratio of a blood transfusion among children with a low preoperative crit, and among those with a low preoperative crit, 28% of the transfusion risk is attributable to anemia. Um, but besides that, anemia is significantly associated with increased morbidity and mortality. This is well known in the adult population, but had never been shown before in, uh, in our pediatric population. So we looked at the Nisquip database, uh, we looked at 50,000, uh, non-cardiac surgical children in, in this NSquip database, and we showed that anemia has a strong independent association with mortality. So this is important, um, So, as I said, there's a plan. It's a work in progress. Uh, it's just an idea at this point, uh, but we, we really need to timely diagnose, uh, postpone surgery if possible to treat anemia, and we can treat with PO iron that may be effective, but there are other modalities, and we're very, very interested in, in exploring IV iron, uh, potentially for those high-risk, severely anemic patients or even uh erythropoietin. So we've talked a little bit about how to optimize and manage the high-risk patients uh by our pre-op and intraoperative management guidelines, but what about those low-risk patients? Uh, so, in our, uh, paper where we looked at predictors of clinically significant postoperative events, there were Uh, we, we designed an algorithm, and I have to thank David Zarkowski for, uh, for this, um, and we looked at whether the patients had any risk factors and accordingly what their probability event, uh, probability of a postoperative event was. So there's clearly a number of patients who are very, very low risk, and those are the ones who Are weight greater than 10 kg, ASA 1 or 2. They are non-syndromic. They don't have anemia. They don't get a a massive transfusion, and they get antifibrinolytics. So we've just started this new initiative, uh, this QI initiative, where, uh, we're going to bypass the ICU and go to the floor. And if the patients meet all these criteria, Um, and they're considered low risk, they, uh, they will start, uh, going to the floor instead of the intensive care unit. The goal is to improve care, decrease complications, maintain patient satisfaction, and decrease cost. And we started this January 2018. Uh, we've successfully enrolled two patients so far, and we've bypassed the ICU and decreased the hospital length of stay, decreased complications, and decreased cost by this initiative. So now in the next 10 minutes, I'll talk to you a little bit more about not specifically what we're doing at Boston Children's Hospital, but more what we're doing for uh nationally and internationally. Um, and so I'm the chair of this group, uh, the Pediatric Craniofacial Collaborative Group. It's a group of, uh, anesthesiologists and surgeons and caregivers who take care of craniofacial patients. We started this in 2011 under the Society of the Pediatric Anesthesia. It's a national database registry of 30 to 35 US, Canadian, and international hospitals. Hospitals, we're growing and growing, so we have over 4000 patients enrolled in this database. So we have the power of big data, um, and one of our first papers, we looked at this benchmarking paper we published this last year, uh, looking at perioperative outcomes and management because it's very interesting to know what's going on, um, all around the country and where we fit in, in, in that, um, and. When we looked at selective outcomes with this group, that I can report that 95% of patients still are exposed to blood or blood products for craniofacial surgery nationally and internationally, and they have hospital length of stays up to 6 days. ICU typically about 2 days. So that's, that's the Metrics, that's the benchmark. And when you look at and you plot all of these 30 institutions and you look at the perioperative blood products, you can see that there's a wide variability between institutions and within institutions as shown by these error bars. Uh, so what's the reason for this, um, and can we improve it when you look at it in, in an. In, in this sort of a, a figure and you plot, uh, there are some institutions that are giving 100 mLs per kilo of blood, so way over blood volume routinely for all their patients. But what are these uh institutions doing down here? Rarely, rarely transfuse these patients for open craniosynostosis surgery. And more importantly, where are we? Uh, where does Boston Children's Hospital fall in this graph and why? And can we improve care? Well, we're sort of in the middle. We have a, uh, according to this, a fairly high, uh, variability in our, in our practices, um, and a And it, we're midway between how much blood products we give. Now, you may say that's because we have very complicated patients, uh, ASC 3 or 4, with, uh, you know, difficult, uh, surgical, uh, uh, techniques, but there may be other things that come into play. And the goal, I think, is to improve care and to try to decrease. Uh, blood transfusion, decreased blood loss, and decreased complications. So, can we use our big database to help, uh, predict and explain any of this variability, improve our care at Boston Children's Hospital, and improve care, um, nationally. There's a couple of more papers that are coming out from this group. Uh, we're looking at predictors of transfusion outcomes, um, and when we look at patients who have massive transfusion, look at independent, uh, predictors of massive transfusion. It's the same as we saw in our single center, weight less than 10 kg, ASA 3 or 4, long duration of surgery, and if the patient, if patients didn't get antifibrinolytics. Currently, nationally, only 45% of patients in the database, when we looked at it, were getting antifibrinolytics. So there's one initiative that may uh improve care. And then we wanted to look at, you know, the patients. With the transfusion-free course because ultimately, that's, I think the goal. And it, those patients that had a higher chance of having a transfusion-free course were, you know, the weight uh greater than 10 if they were ASA 1 or 2, if they didn't have anemia, and if they had a transfusion protocol with antifibrinolytics and CellSaver. So there's lots of room for looking at these things. These are things we can Uh, improve, I think, in our, in our patient care and use this information from our large database to help, um, help optimize our patients. Um, and I spent a couple of hours yesterday in David Zurkowski's office going through, uh, the database. Thank you, David, um, for looking at predictors of complications because we talked about predictors of bleeding and blood loss, but really, does that Uh, can we, uh, also look at complications and does that, uh, amount to, uh, increased complications also? And, um, From this database, there was an overall 13% report of major perioperative complications in these patients nationally and internationally. So still, I think quite a high, uh, number. When we looked at multivariable risk factors for clinically significant uh complications, uh, there were 4 main factors that stood out, and it's the same, the same things. ASA 3 or 4, if they had prior craniofacial surgery, uh, so more complicated surgical procedure, longer surgical procedure. They didn't have an antifibrolytic. Uh, so this is plotting the, the odds ratio for these, uh, and the one that stands out the most is if they had a massive transfusion, they had a 2.5 times increased risk of a major, uh, uh, complication perioperatively. Uh, not only that, ICU and hospital stay were significantly longer, uh, in those patients who had a higher, uh, uh, who had a major complication, so that, uh, amounts to increased cost also. So the goal is really to streamline all this care, decrease variability, and put a nice package or bundle together where we can optimize the care of these patients. And therefore, we've implemented the perioperative surgical home, which takes into account all of these different strategies that we can use to improve care and decrease hospital length of stay. So, you know, in conclusion, craniofacial surgery, uh, can we improve care? Yes, we can. I think Boston Children's Hospital can lead the way. I think we can. Our goal, ultimate goal should be as close to a transfusion-free and more importantly, a complication-free course and a decreased hospital length of stay, course and decreased cost. Um. They're very challenging patients. Uh, we're going to keep implementing quality improvement initiatives for the high and low-risk patients. Um, I'm chairing a, a group to, uh, write some best practice guidelines and evidence-based guidelines for care of these patients nationally because there's still, uh, I think, a long way to go with improving care nationally. And, you know, my thoughts on the goals would be a good patient blood management programs with good anemia management. Um, maybe I can convince Mark and John to wait till the patients are greater than 10 kg if they can to, to operate on them and get their, um, hematocrit up. Uh, maybe we could implement EPO to do that and start, uh, start a program for that. And of course, the, the protocols and the streamlining of patient care to decrease variability. Um, the other thing I'll say is you probably can't read it down here, but if we don't measure it, we didn't do it. So that's something we need to be mindful of doing too. So thank you for your attention. I particularly wanna thank, uh, all the children with craniosynostosis and the patients and parents, all my mentors. I know, um, 15 years ago when I came on staff, Saul Soriano and Um, Neville Seth and Charlie Ngozian were really the pioneers who said, why don't use TXA in those patients, and it kind of got my career going from there, so thank you. I see Saul smiling. Um, thank you to all the, the surgeons, uh, for, uh, letting me take care of your patients and trusting me with, with that care and of course, all the rest of the team, the nurses and the, uh, and the surgical, uh, residents and fellows and anesthesia residents and fellows. Thank you. And Mark, I'd first like to say it's, it's, uh, remarkable to see all the innovations that you've brought forward in the, in the management of these, these kids, and, and, um, I think in this day of, uh, increasing cost containment concerns and what have you, the data that you've compiled already to show how much Uh, more cost-effectively, you're taking care of these problems for the kids is, is invaluable. And I was sitting here thinking this is one of the programs that clearly needs to be, um, promoted for complex, you know, pediatric surgical care to the insurers and the corporations where we're doing it. And I think that, um, you know, the synergy of both of you and the rest of the team working together shows what having folks concentrate on us. On a single problem really can generate new ideas and enhance the care of those kids. I'm sure there are comments and questions from others in the audience. Doctor Rockoff, you're sitting very quietly. Uh, Mark, uh, a question about timing of surgery. How do you make a decision about the age of the patient for the different procedures? Obviously, Susan would like you to wait until the patients are bigger and their hematocrit's higher. My, uh, I, I assume you want to do it as young as possible so the brain growth could remodel the brain. How do you make that decision? Yeah, so, on most points I listen to Susan, but not on that one. So it's um To do the endoscopic surgery, it's one thing I didn't really get, get into, but you're absolutely right. So because we are relying on brain growth to do the remolding for us, the optimal age for that is about 10 to 12 weeks. You know, some might say, well, the optimal age is, you know, date of birth, right? Because that's when you have the biggest growth potential. So there is some compromise, I'm putting in a plug here that I am compromising by doing it a little bit older. Uh, some centers are doing it younger, the endoscopic, they're doing it 68 weeks, but we think it's, uh, you know, we have enough experience now to say if you, if you can wait 8, I'm sorry, 1012 weeks, you'll still get a very good outcome and probably lower the risk a little bit. If you start to get out beyond 3 months, you may not have that, you know, I showed the growth curve at the very beginning. You may not have enough growth to really achieve the correction you want with the minimally invasive techniques. So if we see a baby the day they're born, we'll generally have them come back for surgery at about 10 to 10 weeks ideally, and we do, there is one standard we shoot for, and that's 5 kg. So we find most of the kids are 5 kg at that point. If they're below 5 kg, we may push the surgery out a couple of weeks. Yeah, if you look at that growth curve and what their brain should increase in that first year of life, it's hard to imagine putting it off to a year because of all of that. Logarithmic group, Doctor Soriano. Well, Susan, I want to congratulate you and Petra Mayer for, uh, really taking the lead in the pediatric anesthesia community, both here and internationally for organizing, particularly this group, this collaborative group where you are actually able to teach some of the stuff we learned, uh, we do here at Boston Children's and also learn from them. I think it's an important model for everyone who does clinical research now that, uh, you need to have multi-center trials, multi-center, um, database collection groups in order to be able to show whether the efficacy of what we do is, is true. And It looks like what we're doing is, it works pretty well, and, and we're able to get some insight on how other centers do it. So congratulations to both of you for taking leadership roles in that. I'll just, I, I will point out because Susan did downplay it a little bit, but the study that Susan and Petra and others here did on the TXA was a game changer internationally in craniofacial surgery. It's now essentially standard of care with a marked reduction in, in blood products being administered. And interestingly, there are still a couple of places that like they don't do endoscopic surgery, they don't do TXA and I was speaking to sort of the lead surgeon at CHOP where they don't do it and he goes, oh, there's complications with that. I said, really, you know, we, no one's published any, so maybe if there are, you should publish them. He goes, the safest drug you can give is blood. And I think there's, I think there's pretty good evidence that blood is just not as safe as he thinks it is. Additional questions for Doctor Proctor Goby. Uh, just a quick question about, um, short-term, long-term cognitive neurodevelopmental outcomes. You touched on some differences that are quite large in terms of economic and cost reduction with endoscopic craniectomy compared to the open surgical approach. And Susan's really articulated some important advances in guidelines in the anesthesia management and along with risk ratification. Clear, clear improvements in patient outcomes, but what about down the road? You didn't really comment too much on that. Any perception or any evidence or any ideas regarding patient outcomes in terms of their cognitive development? David, we're particularly endoscopic compared to open if you have some surgeons and anesthesiologists, we don't really care about that, right? I know you do. Um, no, David is, uh, he's, he's leading me on here because he's, he's been working with us on a study we're doing with Yale looking at neurocognitive outcomes and open versus endoscopic. And then, and as David knows and sort of pointed out that the, the analysis done at Yale was very flawed in a prior publication they did on looking at their group where they actually showed their group was in the genius range. So every study on cranial synostosis shows that. The kids are by and large in the normal range, usually about 1 or 2 IQ points below normal, right? So that if a mean IQ is 100, most stenostosis groups are falling sort of 98 to 99 on mean IQ, except in New Haven where they fall at 117. Um, so it's a, it's a pretty flawed study and we've compared our group and our group actually also came out a little too high. It was about 106. So the endoscopic group came out at a mean IQ of about 106, which to me is sort of indication that it's actually, it's pretty protective and it works well and doing the surgery early is, is effective. But as, as David knows, we don't really have great comparison data at this, at this time because The main study out there that looked at the two and wasn't wasn't our patients. They were looking at other groups that were doing open strip craniectomies showed that the open, the bigger operation led to better outcomes, which for a lot of reasons you could imagine how could that possibly be true because the length of surgery went from 30 minutes to 6 hours. The big blood transfusion requirements, etc. much bigger operations, and they were done. Kids that are a little bit older, so the brain had been restricted for a little bit longer. So, we're, we're trying to work through all that, uh, but the, but the main data that we see in cranial sysynostosis surgery is that the kids are generally pretty normal, just tiny bit below normal, and in fact, no, no study to date has shown an effect of treatment changing the neurocognitive outcome, you know, so. For that to be, to be determined, but I would think it'd be more important at, at the Society of Pediatric anesthesia meeting, uh, just last week, we talked about that major initiative that we want to implement for our database is to, is to, uh, get together with the surgeons and improve the capture of long-term outcomes, uh, because, uh, that hasn't been looked at or reported, so. Maybe 10 years down the road we'll have something. But I would think the criteria also would be the important would be the age at which they're corrected, not just whether they're open or endoscopic. Any additional questions? Thank you. I just wanted to switch gears again and ask, does uh tech and rodent point of care testing have any role in the control of bleeding in our protocols? Uh, so, thromboelastography is a way to measure the dynamic, um, coagulation, uh, cascade instead of sending off PT PTT INR or fibrinogen and waiting an hour for it to come back. It's a way of looking at Uh, blood loss at a point in time, and, and, there's lots of centers around the nation that are using thrombolystography. We're using it just for research purposes and we've shown that you can, uh, use better goal-directed treatment, more appropriate, uh, treatment of blood products and limit the amount of blood products. So it's one modality in patient blood management. Uh, that a lot of institutions are using. We only have one machine, as you know, Petra, for research purposes, and, uh, you know, some of the studies that have been done in Europe are very compelling to, uh, to, you know, show that it is very helpful at targeting therapy. But we don't use it here at this yet at this point. But I, I think uh keeping the blood in the patient, then we don't need it, you know, if we can do all these great strategies to uh decrease blood loss, then that more the goal, I think. Well, Susan and Mark, thanks for a very innovative and informative presentation. That's