Stay Current is a multimedia publication designed to keep healthcare professionals up to date with standards of care and new emerging ideas. This chapter is created and edited by Todd Ponsky, Sophia Abdulhai, Abdulruflamoshi, and Rajavendra Rao and is recorded and produced at Akron Children's Hospital in Akron, Ohio. Welcome to Stay Current in Pediatric Surgery. This is Todd Ponsky recording live from Akron Children's Hospital, and today we're going to be talking about lymphatic anomalies. And we're going to try something fun today, something that we probably should have done a long time ago. We are going to have a team of people, experts in the field, giving us the update on where things are with lymphatic anomalies. And today I have a guest moderator, a great friend, and actually someone that I have had such great respect for, Dr. Belinda Dickey. Dr. Dickey is assistant professor of surgery at Harvard Medical School and at Boston Children's Hospital and has really been an expert in A lymphatic anomalies and is going to lead the discussion today. So Belinda, thanks for joining us. Thanks, Todd. So today we actually have the real experts in the field. I'm really the baby in the field, Dr. Denise Adams and Dr. Steve Fishman, who are the co-directors of the Vascular Anomaly Centers here at Boston Children's Hospital. Dr. Denise Adams is a pediatric hematologist and oncologist, and the surgeons of the world know that Dr. Fishman is the pediatric surgeon here at Boston Children's. Perfect. So let's get started. So the session today, we felt that doing vascular anomalies and malformation as a whole was just too much for a podcast and wanted to focus down on lymphatic anomalies, because we get a lot of questions from outside surgeons, outside institutions about what a lymphatic anomaly is and how we manage them as there's been a lot of change in the recent. Probably 10 years. So, I'm gonna start with Doctor Adams, and can you, Doctor Adams, explain to us how the new classification of lymphatic anomalies is? So, the new classification has come about recently, and it's based on a classification system that was described by a surgeon here at Boston Children's Hospital, John Mulligan, many years ago. This classification has been reclassified in 2014 from a group called ISFA, which is the International Society for the Study of Vascular Anomalies. And very simply, the classification in the past was broken down into vascular anomalies or tumors and malformations, but now we know that there are new diagnoses and there are also new genomic testing and somatic mutations so that we can stratify diagnoses that way. So lymphatic malformations are too broken down into lymphatic tumors and lymphatic malformations. And some of the lymphatic malformations are simple, some of them are more complex. So you think of macrocystic, which are the larger cysts, microcystic, which is the smaller cysts, combined lesions, and then there are more significant diseases where the lymphatic malformations can have a soft tissue component, but also bones can be involved, internal organs can be involved. Um, and some of those classifications are a generalized lymphatic anomaly versus a Gorham stout, um, disease. And then also there can be problems with your collecting system. So I think of it when I'm talking to patients as the plumbing, and those are called central collecting anomalies. So there's a lot of new information that's out there. And if anyone is interested in this, the new classification system can be found on the ISA. So that's. ISSVA website and if you go onto the website right away, you'll see a place that says classification and if you click on that, you will actually be able to see the new classification system in its entirety. So Dr. Fishman, classification is one thing. What does that mean clinically when you see a patient because I know you harp on this in our meetings. Thanks Linda. Classification is really important. We've had um many decades of confusion in the entire field of vascular anomalies, in lymphatic anomalies in particular because of using the wrong names. Some of the lesions that we see and treat, we now understand them pretty well, and if we use the proper names, it helps us communicate, it helps us explain to our patients. what the real problem is, and it leads us towards the proper therapeutic options. If we use the wrong names, we confuse ourselves, we confuse our colleagues, and we confuse our patients. For example, we've all been raised with the terms cystic hygroma and lymphangioma. And we don't, if Boston Children's ever use those terms. There is a very, very rare subset where, where sort of lymphangiomatosis word can exist. But in, in terms of the mass lesions that we see, we refer to them as lymphatic malformations because they're malformed lymphatic, just like an interectal malformation is, uh, you know, is is a malformation there or syndacty is, you know, it describes, you know, what's wrong. These are just malformed embryologic problems that happen to affect the vascular system. So if we call a macrocystic lymphatic malformation, Exactly that, that it says what it is. It's a series of cysts that are big enough that you can see them. You can think of them like I often describe them to patients as like a bunch of grapes. You can see each grape, and that has therapeutic implications because if they're full of fluid the size of a grape, you can imagine sticking a needle in, sucking out the fluid, and instilling a sclerosis, and that might lead to the understanding that sclerotherapy. Good option for that patient, as opposed to a microcystic lesion that the cysts are really too tiny to see. It's really a soft tissue mass that is like a small porous sponge, and it's not practical to stick a needle into each one of those little spaces, and sclerotherapy becomes less valuable as an option in most cases. So since Doctor Fishman already jumped into therapy, I'm going to bring up a case, and I would like the opinions of both Doctor Adams and Doctor Fishman on how we would manage that. We're very lucky here that we have an interdisciplinary center where we have multiple specialists look at a patient. And decide on how to treat a patient. So for the two of you, we have a 2 year old who was referred for a truncal mass on the chest and soft, no skin changes on top, um, and an ultrasound was done which showed multiple cysts. What would be your next step? I can go first. This is, this is Denise, the, the medical person, so. I think what Belinda said cannot be stressed enough. I think that we are very lucky here that we have an interdisciplinary team. So it's not one person making the diagnosis and making the plan. It's a group of us working together that's making the diagnosis and making the plan. And so of course, if this patient comes in and From the scenario, you think that it's superficial, and there are a bunch of cysts. But what we need to do is ask about the medical history, is truly examine the patient to see if maybe there are any other lesions in any other place. And then if we, as a team, feel that this is a superficial lesion. With a couple of macrocysts, and it's isolated to one area because we're fortunate in our clinic as well that if a patient comes in and we have a question as to the probably the depth of the lesion or the extent of the lesion, we actually have an interventional radiologist that can do an ultrasound right there in clinic with us. And then we as a team might say this seems very localized, it's a larger cyst that the interventional radiologist can get into, and sclerotherapy might be our best option for that patient, but with follow-up. So Steve, the amount of surgery you do on these malformations, with the advent of more sclerotherapy, um, more changes in interventional radiology, and now new medicines, what is the role of surgery in a lesion like this? Well, if we jump ahead and assume that we've made a correct diagnosis on lymphatic malformation, because Denise emphasized the importance of taking history, and you started with a 2 year old patient, I might want to wonder, is there anything else that could have been, because most lymphatic malformations are present at birth, are visible visible birth. They're certainly present at birth, but most are visible at birth. But it is possible for a 2 year old to present with sudden expansion of a previously undetected lesion. Uh, but you do have to think about the differential diagnosis and our listeners understand the, you know. There are possibilities of, depending on the location of sarcomas and teratomas and infectious lesions, etc. But if we make the assumption that this really is a lymphatic malformation, it is somewhat variable based on the expertise available to you. So we are very blessed here at Boston Children's to have the range of specialists available. We have people who do full-time vascular anomalies for multiple specialties, particularly interventionalology, hematology, oncology, and several surgical disciplines. So 2025. Years ago, we didn't have as many options, and I was doing a lot more straightforward, let's resect as the only option besides sometimes sclerotherapy. Now, we really have a range of therapies and often combination therapies. There's really 4 general options. One is observation and reassurance, which is quite effective for many, many patients. These are not cancers. They're often not dangerous, and we talk about the risks of doing nothing or the risk of intervention. Then there's microinterventional with a needle or a catheter, right? So I wouldn't say interventional radiology. In our institution, most of our therapy is done by interventional radiology, but that's because we're blessed to have 5 of them who do that and have a lot of experience. But there are many parts of the country in the world where they don't have access to an interventional radiologist with such experience, and, and oftentimes it's a surgeon who's doing sclerotherapy, which is just fine. Particularly if they have image guided equipment available, but sometimes ultrasound is all you need, and I still do sclero therapy if I have the patient in the offering of something else, and I do, I do, uh, primary sclero therapy for endo cafeteria and endo luminal techniques. So it's not necessarily the name of your discipline that determines what your access is to that particular therapy. Then there's resective therapy, which is generally done by us surgeons, and now we have really rapidly burgeoning pharmacological therapy available led in the world, mostly um at Doctor Adams's initiative. And so for a localized lesion like this, probably we wouldn't entertain a systemic therapy. Uh, we would probably talk about leaving it alone if the family is so inclined. You'll always have the opportunity to. Intervene later if they choose not to now, removing it or sclerosing it. Our approach would be to give the parents both sets of options, and we would obviously express our experience and our bias. So if the lesion is relatively small and our interventional neurologists are confident that in one or two sclerotherapy sessions that it can have a durable effect that is substantive such that the family's going to be happy with the outcome. Then we would generally go with that. It doesn't leave a scar, it's less invasive. You don't need long-term drains, etc. If the lesion is so small that you can, in a very minor operation, take it off and not, not leave a, you know, concerning uh cosmetic scar, we might also just, just take it off because we know it's, uh, we don't have a promise of one and done, but there's a, you know, we're, we're, uh, more likely to have, uh, a long lasting durable effect with resective therapy than sclerotherapy in that situation. Then there's the very large lesions. So you can have a really large lesion on the torso such as this that can we have commonly seen lesions that extend, for example, from the axilla to the, you know, pelvis, and they can have lots of macrocysts, each one of which is accessible. With a needle and can be sclerosed, but sometimes we will just go right to surgical resection for that because it's gonna take multiple sessions of sclerotherapy, potentially significant radiation exposure for image guidance, and even when you shrink down all those cysts, when it's that big of a lesion, you have a lot of redundant. Skin and you have all the septa between the cysts that when you shrink them all down, adds up to a substantive mass when it's all shrunk down. So you could have a child go through 6 or 8 sclerotherapy sessions and be left with something the family's not happy with and end up resecting it anyway. In that case, we would go right to surgical therapy. It's quite a usually a simple operation if it's extra fascial. Right to surgery versus medical therapy. So now I'm gonna ask Doctor Adams regards to medical therapy. So if you have a very extensive lesion that goes from the axilla down through the torso, um, is large and was present at birth, now I said extra fascia. You said through the torso. I said extra fascia. Well, you're. I'm changing the scenario. Even if it's extra fascial though, what's the role of medicine potentially before surgery, or the medicine after surgery, because this has been a debate before and me having done much more with Dr. Adams versus you who's now coming on later. I would suggest to do medication first and then resection as opposed to resection up front, even extra fashion. So this sort of brings you into sort of a broader question, um, what is the role of a medical provider in your vascular anomaly center? And I think that, you know, in the beginning when I started, what she really means is a real doctor. When, when I first started on this, almost 20 years ago when um Doctor Fishman started, I would go into rooms of patients who have, who had, you know, multiple sclerotherapy procedures, multiple surgical procedures, and I would think to myself, What are, what are the outcomes? Like, you know, what, what are the outcomes of our procedures that we are doing, and what's our follow-up, and how can we change that, and how can we improve on that? And at that time, what we were using as far as medicine for these malformations, was any medical treatment that actually was effective for other things like hemangiomas. And I just felt that, that, that wasn't good enough, because as an oncologist, I really felt that vascular anomalies is an umbrella term, and even if you take lymphatic anomalies, there are many different kinds of lymphatic anomalies that maybe need different medicines, and maybe the medicines really should be geared, as we learned things, on what the specific phenotype and genomic changes were and things like that. So we are fortunate now that we have medicines, and I think Belinda is speaking of one of the medicines, rapamycin, or Serroimus, serolimus, whatever you want to call it. I think the basics to that is that it is an MTOR inhibitor, and MTOR inhibitors block the PICC3CA pathway, which, long story short, has been shown to be a somatic mutation in a lot of lymphatic malformations and also some of, of the more complicated disorders of lymphatics. And we were fortunate to get funding from the FDA back in 2008 to start a study, uh, using errolius with complicated vascular anomalies because this PICC-3CA pathway controls the growth of blood vessels. We did not know. know that PICC3CA was involved and the, and at the time, we had an inkling that it was, and that was our hypothesis that this pathway was involved. But we started using erolimus for the most complicated um vascular anomalies that had a lymphatic component because we really felt that there was basic science evidence that the lymphatics were involved. And that medicine really improved the quality of life. The other symptoms such as pain, improved frequency or decreased the frequency of infections that some of these patients get in the most complicated patients. And so now that medicine has been used a lot. I feel very passionate that it needs to be used in a controlled manner where we actually are following outcomes. We have good supportive care to follow patients through that, and I do feel that if there is a complicated lymphatic anomaly that we as a group feel need some medical. therapy, that if you can get that medical therapy on board before a surgical procedure, sometimes during an interventional procedure or before an interventional procedure, that in general, I think you can soften a lymphatic anomaly, you can decrease the lymphatic anomaly. And I think your surgical procedure is not as extensive as it would be if you didn't have that medicine. Now, with that said, there is a lot of questions that still need to be answered in regards to that. So, that is just our experience, which started in another hospital and and and actually continued at Boston Children's Hospital, where we're basing that on. But there are many prospective questions that need to be answered related to that. OK, your turn. My turn. So I agree with, I, I agree with everything that he said, and I have become a passionate advocate of enter inhibition to determine whether or not interventional therapies, whether they be with a needle or a knife, are necessary, and I have seen quite a number of patients who I in past decades would have operated on a sclerosis where now we give medical therapy alone. I've also become a passionate advocate of medical therapy, even when I know I'm going to do to operate. So I do a lot of, actually most of my clinical career now is surgical debulking of vascular malformations. And I feel that for complex lesions, for example, a patient with Klippel-ternani syndrome, which is not a pure lymphatic anomaly, it's a combined cap lymphatico venous malformation, but many times when I'm doing surgical debulking of a torso or a buttock or genitalia or extremity for Klipoternane syndrome, uh, mostly of the lymphatic tissue and overgrowth. Tissue. I find that the tissue does get softer if they've been pre-treated with erraimus, and because of the theoretical increased risk of infection, I used to make the patients stop the drug before I operated. Now I actually routinely operate without stopping the medication. I'm not necessarily advocating that to everybody else. I can do that under the watchful eye of Doctor Adams. I wouldn't necessarily, Everybody should do that, but it absolutely from an experiential perspective makes the tissue softer, allows me to elevate skin flaps, resect extra bulk, and to have the flaps be much more pliable so that I can get a much more extensive resection with a good closure than before we had serous because the tissue is less woody and much easier to work with. However, in the case that Belinda initially described, when she talked about a macrocystic lesion, which was just on the chest one extra vaginal, we changed it. I, I would not use, I, I don't think, I don't think I would probably, I don't know, ever, I don't like ever or never, but, but I don't, I don't think it's likely that I would find serramus useful for a purely macrocystic lesion. It will shrink it, but it really doesn't matter. In fact, the tissue expansion. Effect that you have on the surrounding skin to allow you to get bigger flaps to close, it's almost easier to operate on them when they're big. So for a purely macrocystic lesion, I probably wouldn't find thyrolimus of value, but for many microcystic lesions and certainly the complex lesions that are, uh, I'm only going to resect a part of it, the part you're left behind, you want to be as soft and pliable and leak-free as you can. No, I agree with you, but you changed the scenario so that I went with it. The other thing that I just wanted to say is that, you know, pre-treatment is one thing, but I think when we see these as infants and newborns, and they start on erolimus, um, immediately almost, that we actually leave them for a length of time before we start debulking unless there's a functional impact or if there's a reason to do so. This is Todd. I have a few questions now. So Steve, I have a question for you. You alluded to mention in the beginning that one therapeutic option is to do nothing at all. Let's take one that is non-isfiguring a small lesion, and you mentioned that it might not have to do anything. A few questions. Number 1, what do you tell the parents? Is the chance of it ever going away? Is it 0? And number 2, You said observation. What does that mean? Do you have them continue to come back to you? Do you have them just come back if it gets bigger? What does that mean, observation? Those are both great questions. Do I ever tell a patient that a lesion can't go away? Well, most of the lesions that we see, I would tell them it's not gonna go away. There are a couple of lymphatic lesions, types of lesions that can, in layman's terms, disappear, although the underlying tissue abnormality may still be present, and. I have seen less than a handful. Of macrocystic lesions, pure macrocystic lymphatic malformations, deflate, empty, and for all practical purposes, go away, and follow them over num, you know, well over a decade and say they haven't reinflated. So I will tell parents that there is a chance of that. Now, that's less than a handful out of probably several 1000 such patients that I've encountered. So it is possible. Now, I can tell you that the most extensive macrocystic lymphatic malformation that I've ever seen in a fetus was one of those that did that. So we did a prenatal consult on a child that had really massive. Cervical and mediastinal lymphatic macrosis to the point where we were worried about airway management at birth. In, in my experience, exit procedures are, are not really necessary for lymphatic lesions. These are soft and compressed. And you can always intubate these children. Unlike teratomas that can be firm and and exit really plays a role. We did a bunch of exits and have really never had a problem getting an airway, but we do plan to be in the delivery room with. Somebody expert in managing the airway when these kids are born. So I remember very distinctly when this, when this child was born and we had really educated the family, shown them pictures, taught them about sclerotherapy, taught them about surgical resection, shown them before and after pictures of other children. This is in the era before Serraimus was was available, and we were in the delivery room and the baby's born and everybody was highly unimpressed. Now it's usually the case at delivery that they're less impressive than. They were on prenatal imaging, but this one was dramatically different, and we did nothing. And then within months, literally less than a year, it was undetectable on physical examination despite having been way bigger than these bullfrog things that that we're used to seeing. In fact, I remember very distinctly in the newborn intensive care unit, the grandfather really was quite vocal in being upset with me because I had frightened his daughter and son-in-law. And I was wrong, and I said, Sir, I'm really sorry that I was wrong, but I'm really glad I was wrong. I said this is a great outcome, but these are very rare children where they really go away. So I think it's not good to give false hope to people. The other instance. Of a lymphatic anomaly that can so-called go away is one we haven't talked about yet, and that is one of the varieties of lymphedema. Babies with congenital lymphedema of the lower extremities can have them regress to the point of non-detection on physical exam, but again, that's uncommon. So for the run of the mill, small lymphatic anomaly, they come to you. And you explain to them the risks and benefits of management, and they say, Well, why not if I just do nothing, is there a problem with that? You say, what? If it's not impinging on the airway, I would say in most cases it's fine to wait. Families are now quite aware of the potential risks of repetitive anesthetics in newborns. Some of them worry about a single anesthetic. And there is some risk to everything we do, so the choice not to do something doesn't eliminate the choice of doing it later. Now, if I'm worried about a lesion that is impinging on the airway, that if it becomes infected or bleeds into it, it's going to cause an airway emergency, then we're gonna say that's not a real good idea to wait. There are also lesions that now, particularly with medical therapy as an option, that we believe early, early treatment probably gets a better long-term result than delayed treatment. For example, microcystic uh lesions on the face, the tongue, the floor of the mouth. We're leaning now towards very early treatment of infancy for those, but those aren't lesions that we would be offering surgical therapy to anyway. OK, and. So those who are being managed either as a delayed procedure or observed, you would you have them come back once a year, or is there not really a protocol for that? Well, as Denise says, it's really good to have these patients on a planned algorithm individualized for their particular needs, and we're really working towards that in our center. Unfortunately, the realities of patients' geography, their family situation, their financial ability to travel can get in the way. That, so we're trying to develop towards individualized management. We, we deal with patients all over the world by video, by photos, by mailing us, uh, uploading imaging to us by email. We have dedicated nurse practitioners who do nothing but vascular anomalies, and they, uh, are quite expert at, uh, assessing and following up patients and helping decide when's the best time to come back. Um, so, but if somebody lived in our neighborhood, sure, we'd like to see them every 6 months or every year for the average patient. So I think it, I think it depends on the, the patient too. If, so if it's a young infant and you have new parents, and you know, maybe you say to them, why don't you come back in a month just to make sure that everything is fine. And then if you see them in a month and things are great, then, you know, you spread that out to 3 months to 6 months, and then spread it out to, to a year. You know, I, I, I think what Steve said is, is right, that I've seen some of these go away and not come back. I've also seen some, like, we, we saw a patient who was hitting puberty and came in with a little effusion and Some other lesions. Oh, and by the way, I forgot to say, you know, my, my son had a cystic hydroma when he was born. Is that important to his medical history, you know? So I am, I am, I am really gung ho on, you know, following so that we know the long term implications and effects of these diagnoses. That's great. All right, so let me ask you a question from the very beginning, Belinda. You presented the patient that had come with an ultrasound. When a patient comes and they don't have a center like yours and they go to see someone like me, a general pediatric surgeon, they usually don't come with anything. Is it? Just to state the obvious, all of these patients should get an ultrasound with Doppler flow, I'm assuming to help make the diagnosis. Well, I think sometimes you can tell a lot by clinical exam. I think the next step, because it's least invasive and doesn't need sedation or anesthesia, is ultrasound. And yeah, we do Doppler flow on them. And then if you still can't tell, our next sort of step is to do an MRI. OK, and for the ultrasound, what you're looking for is macro versus microcystic. Well, first of all, just to make sure it is cystic, macro and micro, and then to make sure it's pure lymphatic versus having some venous component to it, or no? We, we have something that we call Burroughs rule. Pat Burroughs was the endometriologist who was with us for many years in founding our center, and she used to say, you know, if it's not classic history, physical, and imaging. You need to get tissue because although with experience, particularly in a group like ours, we have, you know, many decades of experience amongst many practitioners, you can diagnose almost everything on history and physical, and imaging gives you more reassurance and then gives you extent and. Helps you define therapeutic options. But when we look at a team, at a group, a patient, and we can't quite figure it out, we very quickly go to Burrough's rule and say, you know what, we should stick a needle in that or get a piece of tissue. Now that happens very, very rarely. If a patient's here in person because, because of the experience that we have in our team, but we do do it sometimes and we certainly review lots and lots of histology from biopsies done elsewhere, which appropriately apply the rule at their center where given the experience they had, they couldn't make a definitive diagnosis. We think tissue is necessary with experience in a small number of patients, but we have developed that rule because we've been burnt. I've personally been involved in a, you know, a small lesion on the leg that was thought to be a small vascular malformation, and we watched it, and Pat Burroughs, the radiologist who was the world's expert in imaging of vascular lesion, says, you know, I'm not really sure, and I think this could be tumor, and our expert musculoskeletal. Little radiologist, tumor radiologist said, no, this isn't tumor, this is vascular, and only because it, it thrombosis was causing discomfort, I took the patient to the operating room to resect it. It was a little tiny thing, you know, on the, on the calf, and I got down to it. I'm like, that doesn't look like clot to me, and that doesn't look like a vascular lesion, and I got frozen section and it was a synovial sarcoma. Uh, it was tiny, but it turns out it was metastatic, and the patient ended up. Doing poorly. So we don't hesitate. I've also been involved in, this is not a lymphatic lesion, but, you know, patient that a newborn, uh, who grew and we followed what was thought to be multifocal hemangioma of the liver, which was metastatic neuroblastoma. So, uh, if things aren't classic, we do recommend biopsy, but as Belinda says, ultrasound and MRI will, will make a diagnosis in almost every case if you can't do it on history and physical. OK. When you do present to them the aspiration and sclerotherapy option, what do you tell them, and I know this is hard because it's based on size, but how many treatments do they usually end up needing and how long does that take? So I think depending on the size of the lesion and what kind of lesion it is will depend on how many treatments it will take. If it's very extensive, of course, we sort of direct treatment to the ones that are causing discomfort or pain or issues. That's when if they're very extensive, as Steve had talked about, then you consider doing medicines first, potentially then directing care at certain aspects that are causing troubles, and then you can potentially talk about. The surgery, I would say that some of these kids take upwards of 2 or 3 treatments of sometimes. Sometimes 1 treatment is enough, and then sometimes you need a couple treatments to, to retreat it in a sense. And then sometimes you do 1 treatment and they're fine for years, and then it pops up again and then they need another treatment. All right, can you talk me through what is involved in sclerotherapy? So I don't do as much sclerotherapy just because. Where I trained, I think Steve probably does more of the sclerotherapy primarily. What happens is if we decide that that's the route we take, depending on if it has a lymphatic component or a venous component, really depends on what you use for the sclerosin. For the macrocysts, still, mostly we use doxycycline. For the microcysts, we're starting to use bleomycin, but that actually has to be monitored because of the side effects of bleomycin. And if there's a venous component, then we would use SDS, sodium tetra, sodium tetra sulfate, yes, that one. And so depending on what the malformation or the anomaly is consistent of is what we would use to inject. And essentially, you know, once you get in the cyst, you get with a needle into the cyst, aspirate to make sure. Sure that there's nothing bad. If it's a venous malformation, you have to make sure that it doesn't extend intravascularly. So those ones usually you want to do under pleural and inject with some sort of contrast to make sure it doesn't go intravascular and it stays within the lesion, um, and then you inject. Something I think is really important to a point that Belinda made. If you're doing venous sclerotherapy, and I, and I would say. Uh, other than in the rectum or in very localized lesions in our place, those are almost 100% done under fluoroscopy by our interventional radiologists. It is exceedingly important to understand not just the venous drainage, as to whether you're, you're going to be getting agent intravascular, particularly in places that use ethanol. We, we use very little of that. A small amount of ethanol, uh, systemically can cause sudden fatal pulmonary hypertension. But even with more mild agents, if you have a large venous anomaly, this is something we see typically with patients with Cripplereane syndrome, that you have a big capacitance area of abnormal veins. They will sometimes, but not always, have direct macroscopic outflow to a large systemic vein that can result in pulmonary embolism of the clot you induce with your sclerotherapy. So. It really is essential that you do venous mapping either by MR in advance, ultrasound during, or what our guys do is venography at the time. And if there is a, a large vein that, that has direct connection and doesn't have its own filter effect, sometimes they filter like through the gluteal muscles and they become tiny little vessels before they get back up into the, into the iliacs and the cava, and then it's OK. But if there's a direct pipeline from a venous malformation to the systemic veins, you really have to obliter. Rate the outflow first. So they use intravenous laser simultaneously. They use titanium coils. They use glue. They'll put the kitchen sink of guide wires in there if they have to, but you really have to do that before you induce a clot with sclerotherapy. It's fatal. And like I always say, don't ask me how I know, right? Exactly. So let me back up and say that. You know, our listening audience comes from all over the world and all sorts of different locations, different types of hospitals, from the most high-end tertiary referral centers to remote locations and for someone like me who is not in it like you guys are. My journey has been that I initially 12 years ago we were resecting these and all and then I was introduced to this concept of sclerotherapy and at that time it was so new for us that we just did them ourselves in the operating room that was in Washington. And then when I moved to Akron Children's, you know, here it's done by our interventional radiologist. That movement that I saw from resection to sclerotherapy to now interventional radiology doing the sclerotherapy, is that happening all over, or are there still most centers doing this always resective, or are a lot of people doing the sclerotherapy themselves? I just want to make sure that we capture everyone here because I think there's a good variety of what's out there. I, I think the information about sclerotherapy has, uh, and its value has traversed most of the educated parts of the world, uh, but I think the concept of a specialized interventional radiologist, particularly one that has experienced vascular malformations, is not available every place. And if you, uh, I'm going to make this up, but I'm guessing that if you look at all the patients who had sclerotherapy performed around the world. Including very populous, you know, countries with less resources than the United States and Canada and Britain, for example, I would bet that most of it is done by surgeons because they don't have. You know, image guided fluoroscopy suites, etc. etc. I'm just speculating on that. And again, it doesn't matter what degree or diploma you have on the wall if you have the experience. Yeah, because I, I think you have to stress what Belinda said about, and it all boils down to safety. So there are many different sclerosin agents that are available. Now compared to when I first started doing this as a medical person. And I think you need to have some sort of understanding of what sclerosis you use for what type of malformation, or you're not going to have that safety element, because, you know, these drugs, you know, bleomycin is a, is a cancer medicine. And you know, when bleomycin was first used. For vascular anomalies, there were reported cases of pneumonitis because people weren't watching for that and they didn't really understand that there's a maximum amount that you can use. So I guess the key thing that I'm hearing from this is that really people need to know when to refer and when to get more information. So that was a great discussion on a complicated patient, different ways to treat macrocyst microcyst, interdisciplinary approach, and we just wanted to stress that um there are resources available for people who want to manage these patients and are interested in managing these patients through our center and through other specialized centers. The, you know, the time for referral and the time. For asking for advice, we're always available to be able to do that, and, and we really weren't able to touch on the extent of lymphatic anomalies, and there's much more to discuss, but I think this was a great start to talking about microcystic, macrocystic, and some of the sort of basic lymphatic anomalies that somebody might see in the office. Yeah, I, I would agree. We, we had a good chat about mass lesions, about, about lymphatic malformation as an isolated mass. I, I think it is important to realize. That there are patients who have lymphatic anomalies as a component of conditions which are much more complicated. The patients that even with pure lymphatic anomalies, Doctor Adams hinted at the central conducting lymphatic anomalies, these patients present with subcutaneous chile with uh eroding bones, with chylo cyscites, with chylothorax, with chyle dripping from their urethra, from their scrotum. From the vagina, from their toenails. Literally, I've seen Kyle reflux down and come out from under toenails. Those kind of patients, along with the combined overgrowth syndromes like Clipporiane syndrome and Clove's syndrome, where they have really multi-system and multi-tissue involvement. Those are the patients that are not going to have a simple answer like observe sclerosis medication. Resect that you can just decide in a simple office visit by, you know, reading one paper or listening to one podcast, and those are the kind of patients that we love to be available to give advice and help and to get involved in treatment if other physicians are interested in our input. Is there a number or a website that people can or an email that people can use to contact you with patients they have? So they can either call our office, which is the vascular Anomaly Center office at 617. 355-522-6 and there is an email that also they can send an inquiry to, and that is vascular@children. Harvard.edu. And there's also lots of resources available on our website which is the Boston Children's website which is www.children'shospital.org uh and if you add a slash vascular you'll come up with our vascularomies center. So you know we have a vascular anomaly center here and so I don't see many of these anymore and I'll tell you what, what I can summarize from this number one. There is definitely a wide array of anomalies that we're learning from the classification system. There's, there's all sorts of components of vascular anomalies, and for the, as we're talking about today, the purely lymphatic, you have an ultrasound, may need an MRI, making sure that it's a purely lymphatic malformation, and we then differentiate these by micro and macrocystic as far as treatment. The macrocystics seem to be lending themselves well to either sclerotherapy and medical therapy, but the microcystic ones usually are ones that would need a resection, but often are better treated preoperatively to soften the tissue for a better repair. Did I say something wrong there? No, I think that was a very concise summary, OK. There's clearly so much more with this. This is one of those topics that almost requires 4 or 5 of these. So I think that there's a lot more for us to talk about going into the details of, of the surgical principles and postoperative management, and also other types of vascular anomalies that we will hopefully be able to do in subsequent podcasts. So Belinda, I appreciate you coordinating this and moderating this, and to Steve and to Denise, thank you very much for being the experts today, and I'm sure there will be a lot of questions that will be sent your way from this podcast. Thanks, Todd. Thank you. Thank you. We're happy to help. Thank you. All right, bye bye. We hope you enjoyed this episode of Stay Current in Pediatric Surgery. You can listen, watch, or read all content by downloading the Stay Current in Surgery app. Please send questions or comments to us at staycurrent podcast@gmail.com. We'll see you next time.
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