Vascular Anomalies: Advanced Practice Providers

Boston, Massachusetts we're getting buried with snow today. Um, so without further ado, um, so we're gonna be presenting on vascular anomalies, um, focusing on an introduction to the classification system and also, um, presenting a case, um, highlighting coordination of care. Um, so, as many of you may know, 1 in 3 newborns, um, can present with some type of vascular birthmark. Um, a very common one is a nevus flamius, um, known kind of in the common nomenclature as a stork bite or an angel kiss. Um, and most vascular birthmarks are of no consequence and typically fade with time. Um, the lesions that we typically see are a bit different. Um, vascular tumors and vascular malformations can present for years or even occur lifelong and can, um, cause symptoms that affect daily daily life and require ongoing care, monitoring and treatment. So a little bit on the history and how the classification system came to be initially there were some very, very early thoughts about beliefs surrounding maternal diet affecting how these lesions came to be, eating too many strawberries or too many cherries, for example, and. Also possibly mom's imprinting on their fetuses their longings or fears that if a mom touched her face that um there was a potential for a birthmark to occur there on her child, um, and as we moved um through the history of medicine and surgery, um, a biological classification started to become discussed, um, and this was based more on cellular features and correlating that with clinical characteristics and also history, which, as you'll see throughout the presentation is very important in classifying these lesions. And so the two main classifications that are part of the system now that is recognized by ISA or the International Society for the Study of Vascular Anomalies is vascular tumors and vascular malformation. Oh, OK. So, as far as vascular tumors, these tend to be benign proliferative endothelial neoplasms, um, and tend to be quite dynamic in their life cycle. Um, they are typically not present at birth and can kind of present and grow, um, to a point in life and then, uh, kind of, um, wind back down depending on the growth. As far as vascular malformations, these typically are errors of embryonic, um, development, so they're congenital. They're classified typically based on the clinical and histologic appearance, um, typically of abnormal channels. Um, they are generally sporadic. There are a few that are familial, um, and as time is occurring, we're finding out some genetic mutations, um, that are also associated with these. Um, as I said before, they're congenital lesions, um, but that doesn't mean that they're necessarily noticed or, um, visible at birth. They may not occur until later in life. Um, they can be localized or diffused, and these never involute. That's a big difference between tumors and malformation. Sorry, skipped one. There we go. So this is just a slide kind of highlighting the major categories of tumors and malformations. Um, I certainly won't for time's sake go through all of them. Um, so one of the most recognized, um, tumors is infantile hemangioma, which we will get into a little bit more, um, and also congenital hemangiomas, um, specifically rapidly involuting congenital hemangiomas or non-involuting congenital hemangiomas. And then listed after that are some of the other um more rare um tumors. And then as far as malformations, some of the ones you may be familiar with are capillary malformations, um, more commonly known as possibly port wine stains, um, and also venous malformations and lymphatic malformations. So vascular tumors can be really difficult um initially to distinguish from vascular malformations. So looking at this first slide, um, you know, without having any history, it may be a little difficult to distinguish um if these are similar lesions or different and if they're tumors or malformations. So the photo on the left is actually a lymphatic malformation there on the right side of the baby's neck, whereas on the picture in the right, it's actually a a kaposiform hemangio endothelioma, which is a vascular tumor, also known as KHE. OK, so another example, um, are these lesions the same or different? The photo on the left is actually an infant that has an infantile hemangioma that has both superficial and deep components, whereas the photo on the right is actually a venous malformation also in an infant. So just to briefly talk about our center, um, so we are an interdisciplinary team that is made up of 18 different specialties here, um, we are primarily based on our Boston campus, um, we see patients and help them usually in um a variety of ways. One of them is in clinic appointments, um, that run on the 1st 3 Fridays of every month and all day, um, when there's a 5th Friday. And also just for time's sake and making sure we're seeing patients in a timely fashion, often we um filter specific diagnoses to our specialty clinics um individual practices. We also, um, for the last few years have been seeing patients twice monthly in nurse practitioner run clinics, so this is for some of the diagnoses such as um hemangiomas and lymphedema are two examples that may not need the full interdisciplinary review for care. Um, and another way that we help patients is every week we present an interdisciplinary conference with our team members on Wednesdays, um, again that's on the Boston campus, and this is done, um, with the patients in absentia, so the patients don't need to come to Boston. We have them send. Their um imaging on CD or online to us they send us clinical photographs and either the physicians or families whoever's making the referral, um, sends all of their clinical information, um, medical summaries, labs, medication lists to date. And we review the information and provide our thoughts on diagnosis, treatment options, and possible care planning should they either choose to come to Boston or have specialists closer to home that we can work with and help refer there and we provide those free of charge to patients and they're a great learning tool for our team, um, and then our patient base is primarily outpatient, um, and we serve both national and international populations. Um, and although we do not have a permanent nurse practitioner that is rounding on patients daily on the inpatient team, we try to make, um, consult rounds and daily rounds when, when we're able to. And this is just a slide highlighting all of the disciplines that are part of our center and so we're pretty lucky in having a pretty diverse team that is interested in seeing these patients. So, our case presentation is actually gonna focus on venous malformations today. So I figured I'd give you a little more information on those. So, venous malformations are present at birth, and as I had mentioned before, they're not always visible or evident right away. They are rarely hereditary, although we do know that some families carry a type 2 genetic mutation, so there is one that we know of, we follow that's hereditary, um, and typically venous malformations are composed of thin-walled dilated veins that lack the normal smooth muscles. So as many of you know, a typical normal vein is triple layer, um, that keeps that vein kind of nice and open and the blood running through, um, at a normal pace. Venous malformations have a single layer of muscle to keep it open, so the blood um flows through much slower and it can flow through kind of much more sluggishly, um, and venous malformations can range from simple ectasias, just one vessel that is actually malformed, um, up to discrete spongy masses to quite complex networks of complex channels and can involve tissues and organ systems. Also, some more on venous malformations, um, on physical exam, they tend to, um, often be quite compressible. Um, again, kind of thinking back to that sponge, you can actually really compress it down and as soon as you let go, watch the blood fill back up into the area that's affected often, um, and they can expand with dependency and it's often quite dramatic if you have a patient raise their hand above their head. You can see the color in the bulk drain out of the extremity, and as soon as they put it back in dependency, it fills right back up. Um, they often can slowly enlarge with normal growth of a patient, um, and we often first have patients present or we kind of hear that there's problems, even if it's a known lesion prior to puberty, um, during that kind of growth spurt and hormonal surge with puberty. Um, venous malformations can be associated with superficial clotting. Um, this clotting can be quite painful and bothersome to patients. Also, um, patients can have consumptive coagulopathy, um, possibly requiring preoperative evaluation and involvement with hematology. Sometimes these patients end up on low molecular weight heparin, um, so coagulation screenings are often done, uh, particularly if the patient's gonna have a procedure. And venous malformations can also be associated with limb length difference of arms and of legs. Um, and for some of the more diffuse lesions, patients can have mesenteric and portal venous anomalies, um, particularly, um, in the gastrointestinal venous malformations. So the syndrome that is associated with venous malformations that we're gonna focus our case presentation on today is something called blue rubber blebs nevus syndrome, um, or the abbreviations are BRBNS. So blue rubber bleb um are multifocal venous malformations. They're relatively diffuse. The locations can include skin, soft tissues, muscles, joints, bone, intestinal mucosa, and some organ systems. Um, the skin lesions again tend to be quite painful, um, particularly on the hands and the feet, and again, that can be due to the superficial clotting that I mentioned. Um, GI lesions can lead to intractable bleeding, and we have several patients that we need to talk about intervention that end up being transfusion dependent. Um, there tends to often with these patients be what we call a mother lesion, and I think that'll make itself fairly evident what that means when you see the case. Um, and often some of the treatments we talk about are sclerotherapy, embolization, and operative management, and we'll get into that in a little bit. So this is our case study, um, we'll call this patient Patient F. So his early history, um, at 28 weeks prenatally, um, on ultrasound, he was noted to have a large sacral mass. At birth, um, this lesion again was quite evident. It was 22 by 22 centimeters. Um, he was born a little over 5 years ago. In addition to his buttock mass, the patient was also noted to have multiple 2 to 4 millimeter, um, purple lesions over various areas of his body. His platelets were noted to be under 100,000, and his fibrinogen was noted to be under 50. So initially at the outside hospital that he was born, he was transfused. The referring diagnosis to our center was um a differential of hemangioma, lymphatic venous malformation, and venous malformation. So he was referred to the vascular Anomaly Center here at Boston Children's in his first week of life. We presented him emergently in conference review for obvious reasons. And we, um, with the information that was provided, so clinical photographs, um, medical notes on his birth, and presentation after, and also the imaging, questioned the diagnosis of blue rubber blood nevus syndrome, but also considered venous malformation and lymphatic malformation, and we recommended biopsy on our first pastor and conference. Um, the outside hospital ultimately did not feel comfortable pursuing biopsy, and you could see why that may be the case. So he ended up ultimately coming for a clinic visit here in person in Boston. And once our doctors actually um did a physical exam, they were able to confirm on the physical exam and the rest of the findings that it was in fact blue rubber ble nevus syndrome. And then we went on to talk to the family about a possible plan of care to help um in treatment, and it's really focused on sclerotherapy and surgical resection, um, and my colleagues will get into both of those in a bit. So this is another photo of this patient at birth. And you can see, you know, it, it could certainly be a little confusing. There's, you know, some kind of red staining of the skin with some purple overlying. There's a lot of mass to it, a lot of bulk. This is a little bit different angle. Wouldn't be too difficult to see how someone may consider this is a tumor. Um, and it may be a little difficult to appreciate on the photo, but on his fourth toe right at the tip, he has a very teeny purple to blue lesion, um, and those also are pretty classic for blue rubber bleb. They can be quite small, the rest of the lesions. He also has one there on his left cheek. And this was the mass when we first saw him in person in clinic at age 7 months. So you can see it has enlarged quite a bit as he has grown, which we said is often the case. And this is just a side view, so you can see that there is normal skin abutting right against the side view of this lesion. For the next part, I'm going to turn it over to my colleague Cindy Kerr. Good morning, everyone. It's nice to be here this morning to talk to you about one of our favorite patients. Um, what I'm gonna be focusing on is the treatment goals from an interventional radiology standpoint. In most cases of venous malformation, we attempt to close the venous channel, um, making it more amenable to surgical resection, decreasing the blood loss, and making the margins much easier for the surgeons to get to. In this case, our approach was a little bit different. What we wanted to do was to close what we could, but also to control the bleeding he was having, um, and we used two techniques for that. We use flare therapy and embolization, and I'll have some photos of that for you. And the ultimate goal for this patient was surgical excision of that big mass that we knew he could not have a normal life with. The challenges in this case were twofold. One was the coagulopathy we were dealing with. He was bleeding on a daily basis, and second was the massive size of the lesion and the associated vessels. So I wanna talk a little bit about the pathogenesis of coagulopathy and venous malformations. It's a little bit different than you may be familiar with in other cases. It's definitely not fully understood and it's definitely multifactorial. In the first part of this is that there's the abnormal endothelial cells that Aaron was talking about, the internal structures of the Malformation is not normal. These cells cannot respond to the normal triggers that start the coagulation cascade, um, and this basic. Loss or abnormality is the cornerstone to the coagulopathy that we have to deal with in these types of lesions. Blood flow. Blood flow is essential for a normal response um to trigger the clotting cascade, and in cases of venous malformations, the flow is so slow, so stagnant, that part of the response is negligible. The slow flow also causes more damage to the endothelial cells, the um stasis and the, the blood damage any normal components that might be lingering. The third part of this story is the localized intravascular coagulopathy, and what this means is the blood contained within that large venous malformation that you were looking at is actually abnormal in and of itself. The fibrinogen of that is Extremely low. Any ability of that blood to clot is not present at all. In this case of Patient S, we could do venal punctures, we could do arterial punctures, and he wouldn't bleed any more than any other patient. But if you touch that large lesion, it would bleed in a minute and you would have to hold pressure for an extensive period of time. This is an MRI that shows the lesion, um, pre-procedure, and on the right-hand side is a lesion, just in case you didn't know that, um, and these channels are actually filled with blood that is not moving at all. The only way it moves is if you compress the mass from the outside. So this is what we started with. Um, and in this case, what we know about these large venous malformations is that they're at a higher risk for bleeding than, um, any other types of malformations. The large multifocal lesions are, our biggest concerns in this regard, and serious bleeding complications can be associated with any type of manipulation, i.e., interventional radiology procedures or surgical procedures. So how do we do this? We try to manage those risks and we, um, in cases like this, we would involve our hematology team, but we always get basic labs. We look at the CBC and we wanna know how anemic they are and in this case, Steven had gotten multiple blood transfusions prior to coming and during his stay here. The platelet count, we wanna know what the platelet count is and also the quality of the platelets that are there, so often a blood smear is done. We do basic COAG, PT, INR, and PTT, and this just gives us a basic overview of the coagulation cascade ability. Fibrinogen is our friend, and we always want to know what the fibrinogen is, and this is something we check before, during, and after the procedure. D-dimer is another marker that we use. Again, it helps us give us an overview of the coagulopathy that we might be dealing with. Low molecular weight is something that we use to um Treat patients who are at risk, those patients with the large lesions and the excessive multifocal lesions. And how we use, uh, Lovenox is we use 1 mg per kilogram per hour, or sorry, 1 mg per kilo q 12 hours with adjustments based on response and fibrinogen level. And our usual kind of range that we're looking for for our low molecular weight heparin is between 0.5 and 1 international units per mL. In this case, our 1 mg per kilo was actually closer to 1.4 and 1.5 mg per kilo to help control the bleeding that was occurring. Other strategies that we use, um, mom would have an emergency bag with her, and in it were, was, uh, topical Amicar gauzes that she could use in place right over the top of the area that was bleeding, and they actually worked quite well. Another technique that she used is she got a bedwetting alarm that she would put in bed with Patient S at night. So if there was bleeding, there would be alarm. Um, so that she would be, it would be called to her attention, cause there was potential that he could have a significantly, um, And that was what the technique she used, she would actually tape the monitor right to his body. Um, what, another thing we do anytime we have a patient on Lovenox, we hold that 12 hours prior to procedure. In some cases, um, The Lovenox will be stopped 24 hours, but here we find that just holding it that 12 hours helps maintain the fibrinogen levels in the area in the range that we are hoping for. Platelet count, as I said, is very important and we often give platelets during cases or before, during and after. So that is something that we're very cognizant of, is keeping the platelets greater than 50,000. Bybrinogen levels, we always want those above 100 and even 120 and 150 in cases where we're um seeing. PT we always wanna maintain that within normal limits, and as you know, fresh frozen plasma is used for that. So during our um interventional cases, we would often give multiple blood products to control what was happening. So, as I said, sclerotherapy and embolization were the techniques that were used, and basically, our goal of this was to control the bleeding, to get him to a place that he would be big enough and strong enough to be able to undergo the surgery. Um, successfully. So we did 5 sclerotherapy and embolization procedures over a period of 10 months. So mom was traveling back and forth from out of state periodically for these treatments. At the start of the um procedures, the vessels were noted to be 4 centimeters in diameter, which is much larger. Then we can treat with minimally invasive techniques such as flare therapy and embolization. There are innumerable small vessels just under the surface of the lesion and along the skin um borders, and this is where the bleeding would occur. So oftentimes, the treatment would focus on these areas that were bleeding. We would circle the areas that were most problematic and focus on those. Again, it's all about giving him time to grow and develop. What we used to treat his malformation was everything we have in IR basically. We used ethanol, bleomycin, we use glue, we use sodium tetradactyl sulfate, which is a detergent. And then the final procedure, knowing that he was scheduled for surgical resection in the near future, focused on a deeper area near the left sciatic nerve region, and this was treated in hopes that the surgeon wouldn't have to go in that area and that the sciatic nerve would. And this is just one image that shows you the amount of sclerosis that were used in um it's kind of a drop in the bucket compared to the venous malformation that still resided. So that's my part of it, and Mary Beth is next to talk about the surgical resection. Good morning, everyone. It's nice to be here. We're happy to present. I'm gonna go ahead and talk about the preoperative planning. Um, although we have a lot of patients with vascular anomalies who, um, have sclerotherapy or, uh, operations, um, quite often, it's pretty straightforward and, uh, we don't need to do a, a tremendous amount of planning. Um, for these patients, um, but for a patient like this patient, um, it was an interdisciplinary effort. We started about a month prior to admission and started meeting with various services to talk about what we needed to do to make sure that the patient was, was safe and that we thought ahead about all of the possible outcomes of this and what we would do about those. So we included multiple services, um, including our anesthesiologist, the blood bank, of course. Uh, hematology, interventional radiology, we have a wound care nurse practitioner, um, who was involved in the planning. Um, the critical care team was with us, our general surgery people, uh, all of the, um, OR, uh, both the physicians and nurses and techs, uh, are involved in planning our vascular anomalies center and cardiology. The issues that we thought were going to possibly going to be problematic that we wanted to be sure that we had thought through before this happened was, as Cindy mentioned, his consumptive coagulopathy. He'd been on chronic low molecular weight heparin since he was. 6 weeks old, so the thought was in planning that what we would need to do is we would need to admit him to the hospital, um, a day or two before the operation, start doing the labs, and transition him over from the, from the Lovenox to the heparin. Um, also, a big concern was, as you could imagine, the size of the lesion and then the blood volume that was within that lesion, um, so he probably, they sort of estimated just by looking and thinking this through that the blood volume in the lesion was greater than the rest of his body. Um, so they started thinking about whether it would be possible to try to move some of the blood that was sitting in that really big buttock lesion out of it before they did the operation that would decrease intraoperative bleeding and his other risks of, of, um, exsanguinating on the table, essentially. Um, they thought about using some CBP monitoring and phlebotomy and trying to move blood out of the lesion into his circulation and then into the cell saver for use for um. To transfuse him during the operation. Uh, also just intraoperative blood loss, period. The, the initial request was for 75 units of packed cells, 30 units of platelets, additional FFT and precipitate. They also talked a little bit about whether what they could do once they had, uh, decompressed this lesion of a lot of the blood in order to keep it from refilling, um, before they tried to, um, to resect it. So there was some discussion about placing sutures and pledges. At the base of the lesion just to compartmentalize it once it was decompressed. Um, and then they talked about worst case scenario is if, if, if this plan didn't work, um, what they would do, would they need ECMO? Would they need bypass? Would they need to do circulatory arrest in the OR in order to keep him alive, um, during the operation. They also talked a lot about positioning because of the size of the ttic lesion and then what was gonna happen postoperatively with the wound that was, uh, gonna be the result. Um, he was initially admitted to ICU. They wanted to monitor him first of all, get the laboratory studies done. They wanted to transition him to the unfractionated heparin. He had a cardiology workup, um, a chest x-ray, EKG, and echocardiogram. His echo actually only showed mild, uh, mitral and tricuspid regurgitation. Otherwise, function of his ventricles was normal. Um, we also had hematology and nutrition consultations, and they took him to the OR, uh, a day before the operation and placed as many big lines as they could so that they could attempt to do this, this decompression of the lesion and just have, you know, if they're going to pump in blood products, of course, you know, they're going to need really big lines to make sure that, that they can do that. So this is just a picture of intraoperatively. This is positioning in the OR, so of course he's prone. They've got him padded as appropriate. They've got the lesion exposed um on his buttock. Sorry. OK, so the, the really interesting part of it was that they, there were multiple surgeons. Doctor Fishman was the primary surgeon, but there were, he had lots of hands in the OR. And they actually spent the 1st 1 hour in the OR compressing this um vascular, this venous malformation over a 1 hour period, and what they would do is they're manually compressing it to push the blood into the central circulation. They're watching the CBP. They're doing monitoring to look at the pressures when the pressures reached a certain point. Then they would remove blood via a catheter in the right internal jugular in order to, you know, get the blood out, keep his blood volume at a more normal level, keep compressing the lesions, so it took quite a bit of time. They sort of maintained pressure on it to hold it down as they slowly move the blood out, um, and push the blood, uh, you know, through this catheter into the cell saver for use later. So at some point they got about 80% of the mass decompressed. You can see it looks pretty floppy, um, and then at that point, once they had a lot of it decompressed and they were comfortable, they thought that they could go ahead and proceed with the operation. They did some, um, suture ligation of the mass with these pledge did sutures all along the, the edge at the bottom, certainly to, to close that off then so that, um, it wouldn't refill with blood. And then they resected the mass. So this is usually just some intraoperative operative photos that were taken. They took many, many photos, but these are a few of them where they resected the mass. And then closed up the wound and applied a back dressing, and what they do with the back dressing is just applied it all to the outside of the entire area to maintain pressure on that area so that it wouldn't refill with blood during the course of his treatment. Um, they figured that total blood loss was about 5 L, phlebotomy 2 2.25 L, and then I listed out just, you know, the estimate was, um, certainly much greater for the than the blood product that would be needed, but they, they actually did pretty well with using the cell saver and then units of pack cells and platelets as needed. So post-operative care, he went back to the OR multiple times over the course of his time in the ICU for debridement and changing of the back dressing. They also did the final wound revision in the OR. He was in the ICU for 2 months, uh, postoperatively, finally was transferred to one of the surgical inpatient units. And then he was only there one week before he was able to be transferred back to the local hospital for a one night stay before he could be discharged home. So I think, um, you know, we, we accomplished what was hoped that he would do well during the surgery, that he would survive the surgery, and that then he would, he would thrive um once he healed and recovered. So this is an example for us of a really high risk patient who needed a lot of services with multiple team members here so that we could get him to this point that you see him now. He's now 3+ years out from his operation. He's walking. He's doing well. He's doing all the things that a 5-year-old should do into school, so we think. You know this is a success for us. It's an interesting case and it took a lot of people working together to get to this point, but it certainly is a major success. Um, I, so at this point, we, um, we wonder whether there are any questions for us about vascular anomalies in general and this case in particular. Donna, is it OK if I, uh, this is Todd Ponsky. Do you mind if I ask a question? I don't know if Donna can hear me. Um, can you guys hear me out there? Yes, we hear you. OK, perfect. This is Todd Ponsky. I just have a question. First of all, thank you for that talk. I can tell you what I learned from that talk is why we don't do these cases and send them all to Boston. This is clearly, I think what everyone just saw here is not the norm. This is, these are unusual cases of vascular malformations that The stuff we see every day are probably the more small lesions that uh we deal with on a day to day basis in the outpatient clinic and sometimes they come in uh as an inpatient. Um, I'm just curious both for those in Boston or anyone, and I'm, I'm asking, uh, Stefan, can we open up the phone line and put the number for the phone line on the site here? I want to invite everyone to call in their questions, uh, if you want to actually call in, um. I, I, I just wanna, uh, just curious to poll the audience, who here, you know, we see these uh vascular, the, the lymphatic malformations, that's very common. They, they almost look like lipomas, but you, in kids for whatever reason, they're, they're, um, venous, they're uh lymphatic malformations and, um, lymphangiomas, and I was taught to resect them, and I know you talked here about sclerotherapy, and then in my fellowship training, we started sclero using sclerotherapy. Um, and I'm curious, um, number one, to pull the audience, who, uh, at their hospital, do the surgeons use sclerotherapy versus resection, um, or, or see how the question, do you use sclerotherapy? I'm just curious. It looks like 78% of the audience at their hospital, sclerotherapy is used instead of resection. I think that's a new trend. Are you finding um There in Boston, are you finding that that's becoming more of a trend for the even the small routine lymphangiomas, or is that an exception? Um, I think we, we sometimes estimate about 75% of the patients that we see in clinic or present in conference who end up coming to us that the first line treatment is sclerotherapy. It's much more common than surgical resection, certainly. Great. And is there, I know for us, you know, we, we selectively do them, um, um, and I know we have, uh, Dean Anselmo on the, on the phone here. I don't know if he can hear us, but, uh, uh, I know that if it's macro, so we look at the, we get an ultrasound and if it looks like there's, if it's macrocystic, uh, then we'll do sclerotherapy, but if it's a bunch of tiny little microcystic things inside, then it's going to be a harder thing to use sclerotherapy. Is that the same thing that you do there in Boston? Yeah, uh, we actually use bleomycin for the microcystic lesions. Um, and, uh, you know, there's always the definition of what a microcystic lesion is, and we, we say anything that you cannot put a needle in is microcystic. Ah, so then, um, and I guess, and who does it there? Is it the surgeons or the interventional radiologists? It's interventional radiology here. OK. I'm wondering where do you do it yourself as a surgeon? I, well, it, it depends what hospital I'm at. Um, but, uh, when I'm here, I, uh, I do it myself. I, um, uh, I basically withdraw in the operating room under ultrasound guidance. I withdraw the fluid and then I inject doxycycline. And then I wait 30 minutes and just suck it back out. And the reason it's hard with the microcystic is you put the needle in and you might suck out one little lesion, but you probably have 50 more to suck out and so it's kind of challenging to get in that space. But I think the key point here is that this is a common thing. Um, the lymphangiomas are probably the most common, at least maybe not in Boston because you see such complicated things, but I think for the routine for, for, for those who don't have such massive vascular. Malformation clinics, um, we see these small hemangiomas, uh, these small lymphangiomas, and always the question is, do you, do you operate? Do you leave them alone? If you do treat them, do you surgically resect or do you put, uh, do sclerotherapy? Um, and I'm just, I think that that's sort of the more common thing that most of us will deal with when it comes to vascular malformations. I think, um, uh, Dean, can you hear us? I see him. I don't think he hears us yet though, so um. I don't know if anyone else is Todd, can you hear me now? Hey buddy, how are you? Good. How are you? Good. Oh yes, go ahead. I'm Dean Ensel. I'm, uh, so I'm a pediatric surgeon at Children's Hospital Los Angeles, and I'm co-director of our vascular Anomalies clinic here. Um, and we, we have had a VA clinic for about 6 years. So, um, Todd, to answer your question, yes, there is now a growing trend to treat macrocystic or combined lymphatic malformations with sclerotherapy, and our agent of choice has now, uh, become doxycycline. We're seeing, uh, kids with very large macrocystic lymphatic malformations that after 2 or 3 sessions with sclerotherapy, they really just deflate numb, and they can have a very, you know, long durable response to sclerotherapy, um, the, for the very small ones, like you said, surgical, oftentimes surgical resection, you know, we can, we can do that very easily with um. Um, and effectively as well for the smaller microcystic lymphatic malformations, we also do use, uh, bleomycin, particularly for the periorbital or retroorbital lymphatic malformations, um, but again it's a, it's a combined approach, um, and if there is residual component of the malformation that's either, uh, causing some sort of, um, deformity or symptoms, then we will, uh, perform a, a type of debulking resection. But these, these can be very tough cases, um, and, uh, as far as the case in Boston, that was a beautiful illustration of, of the necessity for a multidisciplinary approach with these vascular anomalies. This is not the vascular anomalies, it's a very good example of, of a problem in kids that really requires, you know, multiple specialties, um. To be involved in the care, particularly for these, you know, these complicated, uh, large venous malformations. And, uh, one thing I, I recall Steve Fishman saying at one of the best, at one of the ISPA meetings is you do, you really do have to be prepared for, um, considerable blood loss, especially with these, with these large venous malformations. Um, I am curious though about the approach that was initially taken to. Essentially compressed. It never really, I mean, we, we've developed quite a few large vascular malformations and, and I, I'm, you know, we're always prepared for. Um, considerable blood loss. We've had some where we've replaced the blood volume in a patient at least once or sometimes even twice during the course of surgery with, with the use of Cell saver, but, uh, never really considered compressing the lesion for a period of time and then essentially performing phlebotomy, um, do, do you think that that. Reduce the total blood loss in the case or made it somehow, um, you know, less risky or was it um. Does Doctor Fishman feel that that was, you know, that reduced the total blood transfusion need in this case? Um, I mean, it's, uh, I, I think he would say that all of those things are true, that it certainly did decrease his risk of bleeding and decrease the, the, um, the, um, products that were needed for, um, transfusion. Um, I think that idea actually the decompression and the CBP monitoring was, um, that sort of came from the anesthesiologist, um, initially who works a lot with Doctor Fishman on these big cases. Um, it, it's, as far as I know, it's not something that has been done before this patient and um haven't needed to do it since, but, um, it certainly did really work well for this patient. Just to add to what Mary Beth said, I think the other piece they had talked about quite a bit during the planning meeting was they were quite worried about the rapid fluid shift and the burden on his heart. I think that was the other piece too, why they kind of came up with that plan. Yeah, especially that, you know, it's a posterior lesion and the patient's going to be prone for a long period of time. And as you've mentioned with venous malformations, the blood drains out. If you, you have an extremity with a venous malformation, you raise it up in the air, all the, all the blood kind of drains back into the body, so. Um, so I, I certainly, I'm not surprised at the outcome with, with your team and with, with Doctor Fishman, and it's uh, it's a very, uh, very good case and a great outcome. I, I, I think, uh, I, what's exciting here is we got Dean on the one coast and we got the group here in Boston on the other coast. Uh, so we have great resources. I, I can tell you what I reaffirmed is that we, that In a case like this, I think, and I, I probably don't speak for all pediatric surgeons, but at least for my practice, this is best handled by someone who deals with this a lot. And especially with the mass, I mean, exsanguination that happens with these, uh, the potential risk is pretty high. And uh this multidisciplinary team that you all have demonstrated there in Boston and Dean, what you described there in LA is so critical and unless you have that multidisciplinary team. Uh, if this can be a real challenge to treat. And so let me ask you both, could you, uh, in the chat box there, um, if you wouldn't mind writing either your email address or some way for us to contact, uh, you all if there's a, a patient we have with a question or a patient that, um, uh, may be better served, uh, in your hands than ours, it would be very helpful to, to have that, uh, resource. Yeah, I would be happy to, you know, uh, Boston has their, their Wednesday, uh, conferences and frequently I, I get emails from all over the country and, and actually just, you know, from all over the world with again pictures and, and we sit and we don't have a formal conference, but we, we do, um, provide advice and input into difficult cases so we would continue to be happy to do that. That's great. The one thing I'd like to point out is that when we just looked at our database, over 60% of the patients that are referred into our VAT clinic in Los Angeles come in with the wrong diagnosis, and at times the wrong treatment has already been initiated on those kids. So it is very important to get them to the right place and also to continue to educate the public so that these things can be better identified and the kids can get, um, optimal care in a timely fashion.