All right. And just so that everyone's aware, we are recording this meeting. I do anticipate, um, Probably ending a little bit early. Um, but, uh, um, Anticipate a good discussion this morning. So, uh, I've been asked to, uh, talk a little bit about, uh, vascular malformations and tumors. Um, I prepared a, uh, a combination of, um, Uh, questions, uh, from the resources that are available to us both on the score website as well as, um, uh, through the, uh, pediatric surgery, not a textbook. Um, Uh, information. And, um, my thought on how to do this this morning is, uh, basically we'll do this as a a collection of questions, um, and, uh, hopefully some discussion that, uh, branches off those questions. Um, so, uh, Vascular malformations and tumors. Um, uh, two quick comments on these. First of all, uh, You know, vascular malformations and tumors range from uh clinical entities that are Basically clinically irrelevant um to uh complex, potentially and probably um uh unsolvable problems in other circumstances. Uh, and so the, the range here in terms of the, uh, clinical relevance is very wide. Uh, and also the, the nature of these issues is, is also quite wide, I think. Um, we're gonna start off with, uh, the first question. Um, uh, so this is a, uh, question 1.1, uh, and, and this comes directly out of the score, um. Uh, information. Uh, here, here is a, a, a 1 month old female presents to your clinic with, uh, multiple cutaneous lesions. Um, uh, these lesions were not present at birth. They have, uh, uh, continued to grow and increase in number since they appeared two weeks ago. And, um, uh, I, I found a photo on the internet. This is not off score. This is my personal edition. Um, uh, I, I, I found a photo of this off. Using Google. Uh, but this gives you a sense of, of what these lesions look like. Um, and so my first question is, What could this be? And I, in terms of answering these questions and kind of But leave that up for, for volunteer. Um, but, uh, this lesion here, you've got a one month old female. Uh, these lesions were not resident birth. They are now there. They are increasing both in size and number and, um, That's one of them. What a week. Sure, go ahead, cherry angioma, a cherry angioma, OK. The this has a, it turns out, yeah, these have a bunch of different names. Uh, cherry anginoma is actually one of them. Um, although I probably would have picked a different fruit, but, um, uh, that's OK. It is, it is a, a red fruit of some kind. It's good. Um, OK. Any other names for this? What's that? Yeah, infantile hemangioma is another name for it. OK. So, uh, yeah, historical names for this. Um, strangely enough, do two of them do include, uh, well, actually many of them include a fruit, not a cherry, but it happens to be a strawberry. Um, a strawberry mark, strawberry hemangioma, strawberry birthmark, uh, a strawberry nevus, um, or even a, a cavernous hemangioma. Um. Uh, the reason for these developing is Relatively unclear. Um, we don't know. Uh, they do appear to run in some families about 10% of the time. Um, uh, there are a few cases where they're associated with other abnormalities, um, uh, such as the, uh, face syndrome and the diagnosis generally based on, uh, the symptoms and appearance. So What characteristics of this presentation make this most likely to be an infantile hemangio or strawberry mark or Yeah, one of the most important things that wasn't present at birth. Um, so for We think of vascular malformations is different than uh hemangiomas. Those are present at birth or congenital and grow with the infant. This is rapidly growing, uh, it wasn't initially seen. So the both the growth velocity and the presentation time-wise would be two things that would be suspicious for it. Excellent. OK. Yeah. So, um, uh, these are some of the examples. Very good. So we've got, uh, they appear as a red or blue wa lesion. Um, they're not present at the beginning of life usually and, uh, then they develop between in that first month. Uh, they tend to increase in size for several months, uh, and then, uh, uh, tend to spontaneously shrink in size and disappear over the next few years. Um, uh, however, depending upon their size, larger they are, uh, they can lead, uh, some, uh, durable, um, skin changes after they shrink. Um, they, uh, can have, uh, some symptoms including, um, uh, pain, bleeding, ulcer formation, uh, disfigurement, and, and then in extreme circumstances, uh, uh, some heart failure. Um, most common, uh, these are the most common, uh, A tumor of the orbit, periorbital areas, children, uh, uh, in childhood. And then, uh, of course, these can occur, um, on the skin as we discussed. They can be slightly subcutaneous, uh, they can be on mucous membranes, uh, of the oral cavities, lips, um, and, um, extracutaneous locations specifically, uh, the liver and, uh, GI tract. All right. So one more question. So in in terms of um Uh, these lesions, um, are there any imaging studies that should be obtained? Um, so I think it depends on the number of cutaneous lesions, um, I think. Textbook said like 6, if you have 6 cutaneous lesions, and ultrasound of the uh abdomen would be uh Fruitful and making sure there's no abdominal. Uh lesions as well, but if it was just a small number. 2, and I don't think I would, but I think the number that I read is 6. But obviously, the, the ultrasound there, exactly right can be helpful. Um, and then, uh, uh, this I think is uh an important thing to, to understand. So for this patient, um, what treatment options are there, uh, available and, uh, would you recommend them? So I think, I think there's a few, uh, the first would be watchful waiting. Um, these will, uh, as we talked about, will evolute. Um, I think historically steroids used to be used, but propranolol has become the Uh, medication of, of choice. Um, I'm not sure I know exactly when I would just wait and watch and when I would, um, opt for medical therapy. And then the last one which, uh, it would be rare, I think would be surgical excision. Um, Uh, but those would be in cases where there's concern for the actual disfigurement, uh, from the lesion itself would outweigh a surgical scar. Yeah, I, I think, um, uh, that's exactly right. The, the Indications for treatment are, are really dependent upon where these are, how big they are, how much they're, uh, what kind of long-term consequences they may have, and if they do have any, uh, current symptoms. So, uh, as you mentioned there, kind of a, a review, in most cases no treatment, uh, is needed other than just observation. Um. There are circumstances where they may result in problems, um, and of course, uh, medications propranolol, uh, and steroids are recommended and occasionally, um, surgery and I guess there's some laser treatments, uh, for some of the containing solutions that we need. OK. So I guess I would just end up to say, I, I think of all the vascular malformations, this actually has options that require you to think and then just cut it out. And they went through the whole stem of my oral board exam, uh, including what dose you're giving, how you're gonna monitor the initiation propanol therapy, how you gonna uh the. response. So I would have a thoughtful process on whether there are hard and soft criteria you're gonna use to either observe or watchful wait. I'm sorry, observe or um stop propranolol, and then when you're gonna consider prop success or failure, when you're gonna upgrade. I think that there is actually evidence this. Was laser considered an option too, or they, they gave me a head and neck one that was growing and then causing airway problems. And, and I think that's the critical issue is uh in, in terms of Surgical intervention, um, There really needs to be some kind of impending, uh, Physiologic issue, um, such as an airway problem or for whatever I exactly that's right. And so this one actually is, um, a liver one, right? So we're gonna talk about the, the liver here just a little bit and, and get into a little bit of the detail about, um, uh, the propranolol and some of the other things to look out for. So, um. Question number 2 here is you've been asked to, to evaluate a 4 month old child with multiple liver lesions and signs of congestive heart failure. All right. And uh this happens to be an ultrasound again not providing a score. It's is something that I, I pulled off of uh using Google but it is a uh ultrasound of the liver um uh with uh flow uh enhancement, uh, flow color enhancement on the right side. Uh, and, um, give me a sense for what we're looking at here. Possible arteriovenous malflammation. Yeah, you got some liver and there's blood flow in, in multiple directions and it's actually Kind of larger in size, right? So you would expect blood flow in the liver normally, um, but these, uh, uh, flow areas are, are, uh, larger in diameter than you'd expect, uh, and so the, the, the concern here is that this patient probably has, um, well, I guess I would say, uh, what, what do you think this could be since we've been talking about this. It could be amazing. Yeah, so this, this is, uh, as kind of we're discussing, we're talking about, uh, infantile hemangiomas, skin, uh, face, um, subcutaneous, uh, head and neck, but they can also occur elsewhere, uh, and this could be, uh, all over, um. Human. So, uh, how are these, uh, how are these classified? I guess I'm giving it away. Um, so they are, uh, classified as, uh, focal, uh, multifocal or diffuse. Uh, that's images there. These are not ultrasounds obviously. These are MRI's, um, and, importantly, uh, the, one of the things that the, um, uh, Uh, score information as well as the not a textbook, uh, information points out clearly is that, um, When you've got a focal lesion, um, these are basically the adult type, uh, hepatic hemangiomas. Um, whereas, uh, infantile hemangio, hemangiomas, um, tend to be multifocal or diffuse. Um, And here you've got a, a, a, a vision there what a focal one might look on, look like on MRI on the left, uh, multifocal in the sound and diffuse on the right side. So What causes the uh congestive heart failure that we see in this patient and, and, and in these lesions and uh how should we managed. So the, the pathophysiology is a high output, uh, heart failure. So basically it's a shunt running through the hemangioma that uh increases venous return. So the systemic system. Um, so depending on, uh, so this patient specifically, uh, As far as workup that we can confirm the diagnosis and there's no other sources of the heart failure. Um, I think it'd be managed in a couple of ways. And it, and it depends on if this is, this is diffuse or Focal, multifocal. I mean, uh, in a, in a focal lesion, um, uh, consultation interventional radiology for embolization, uh, it's possible if that's uh not successful, you can, uh, surgically resect the lesion if it's causing significant heart failure. Uh. Uh, for optimization, uh, those options are not, uh, or not the Always feasible for diffuse lesions or multifocal lesions. The second one was. Is there, uh, is there a role for, um, therapy. Um, I think both with the, uh, management of the heart failure and I think beta blocker, uh, beta blockers as well. Yeah, yeah, exactly. So, um, with these hepaticamia, the these are caused by microvascular shunts, um, and, uh, still the treatments here are, uh, steroids, um, and propranolol and then embolization, um. And I think this is actually an important point that um not a textbook actually pointed out very nicely is that these are not believed to have malignant degeneration, um, which is actually not what I remember, um, it's, uh, at, at Utah Valley years ago um we had a, uh A couple of twins. Um, one of them had, uh, a fairly large, uh, infantile hepatic hemangioma. Um, With some, some degree of significance shunting. We did not need to embolize, we did not need to surgically resect but we did start propranolol um and the patient did get steroids. Um, and, and the management of this, uh, a baby's propranolol was fairly complex because these's parents lived in, in Saint George, um, And uh James Hoffman, which some of my partners will remember, uh, the cardiologist at Utah Valley was kind of managing the propranolol, uh, we got it started and the newborn ICU we hadn't had this baby on propranolol for years. This was not a three-month thing or a six-month thing. This was a, uh, a multi-year, uh, treatment period for the propranolol and, um There was a question as to whether or not we should be checking these for uh malignant degeneration. Um, and so we were getting, uh, Alpha fetoproteins uh in this patient, um. Uh, once a year, every 6 months for a while, and then once a year for a few years, um, so about. At the age of 4 or 5, but I don't believe that's the case anymore, um. Is it Is anyone getting me following these for generation on the? All right. So steroids, propranolol, embolization, um, and the, the details of uh the dosing of the propranolol, um, Uh Yeah, I'm not gonna address that, but, uh, the embolization can be very useful in some circumstances. OK, just say a couple of things on that 11 in terms of steroids, I, I don't, hopefully nobody's gonna expected it. I think the important thing is they can become hypoglycemic. Right, because the propranolol and the propranolol mask the symptoms, and monitoring for hypoglycemia is an important part of mission propranolol therapy. And kids with hepatic hemangiomas are also at risk to get hypothyroid. So that's what for check. From the propranolol, yeah, or I don't recall, but it's that's something that we do. Excellent. OK. So we got uh, a, a neonate uh presents with reddish purple, purple lesions on her trunk. Um, and again I found a, a, a photo on Google, um, and, uh, it's an example of what we might be looking at there. Um. So, I guess the question is what could this be? And this one's now, we've gone from uh infantile hemangiomas, strawberry marks, um, and hepatic uh hemangiomas now to something that I have actually not seen much of in my career. These are not. All right. And I guess the next question would be, would you want any labs in this patient since we, we don't know what this is. Um, if you have a counsel for this, what labs will you wanna surely got? It's certainly a CBC, OK, CBC. Anything else? I'm not sure specific to this, OK. All right. So we're gonna be spending a few minutes talking about uh Casa Bach Merritt syndrome. Um, So again we've, we've gone from something that's relatively common um to something that I'm not sure I've ever seen clinically in my career. Um, um, nevertheless, uh, this is a, a, a, a key item, uh, identified in, in score and not a textbook so we'll spend a little bit of time talking about this. So yeah, your CBC is gonna be helpful here, um, because you're looking for thrombocytopenia, uh, particular, um, less than 50,000, um. And then, uh, uh, these patients tend to have what's called, um, a mic microangiopathic hemolytic anemia, um, and a consumptive coagulopathy. So, So we should take a moment and just kind of describe what this uh uh Casa Bach Mitt phenomenon is, um, and uh the vascular tumors that are associated with it and uh the imaging findings, uh, we expect to find and then, and then what treatments are available. So, uh, these, um, are the, uh, kaposiform hemangio endotheliomas. Um, these tend to be larger than 8 centimeters, uh, and they are enlarging vascular lesions. Um, uh, they tend to present early on in life, or, uh, early infancy. They tend to be a single lesion, um. Uh, as we've seen on the photo, uh, they can be red and purple. They're tense. They're edematous, uh, they're, uh, uh, both, uh, cutaneous and subcutaneous. They're warm, um, and I think historically, uh, there's been, uh, even a tuft of angioma. So, What imaging findings then uh would be consistent with a, uh Kaposiform hemangio. End of the. Stemming from a vessel, I'm not sure what the See an ultrasound or MRI. OK. So ultrasound Or MRI, um, that's right. So these are, these are MRI's um that we use to, to, to characterize these and you know You know, despite the question asking for uh specific findings, I think that the important thing here is that these tend to not have specifics. So you have kind of ill-defined margins on imaging. They tend to not respect tissue planes, um, and they tend to be very obvious visually when you see the patient. Um, so for these, uh, uh, the, the treatment options, um, Get a sense for what these would be. Well, it's certainly the uh uh it's good, depending on where it is, it could be a very difficult resection. Um And so I think, I think to start it would be uh uh optimizing the patient's clinical status and coagulopathy as well as um I wonder if this would be a treat amenable to steroids as well. Yeah. Um, so generally speaking these are not surgically resected. Um, uh, they, the extent of them, the size of them, they tend to violate, uh, uh, pass through multiple tissue planes. Uh, as a general rule, we're, we're not going in removing these. Um, but your main treatments here are corticosteroid. As you mentioned, uh, and then Ben Christine with interferon. Um, and Eric may want me to discuss dosing of these two things. your dose. Um Corticosteroid that help by chance, uh, but the increased interferon. So the, the question is, you know, in terms of uh uh relevance for, for our, uh, uh, discussion and, and for your, um, uh, for studying for pediatric surgery boards. Um, obviously, you know, hepatic hemangiomas, you need to know definitely, uh, about how to do those, um, head and neck, infantile hemangiomas definitely, uh, knowing, knowing how to dose, uh, the corticosteroids and propelol is probably very helpful. Um, This is pretty esoteric, I think, um, but probably useful to know. The big thing to know about interferon that was commonly used before propranolol was, was recognized as being effective and, and it's complicated by spastic. Not a small number, so that's probably. The one thing about it. it's good. And so that's a big decision when you go forward with that. I remember it's, uh, I mean that it's a really poorly tolerated medication. So trail. And is that are going back to the infantilangioma is, I know propranolol's first liner is steroids still being used? I kind of, the chapter seems to suggest that it's kind of faded away for some of those who's been so successful. Propranol is very successful. My, my guess is, uh, steroids would be kind of adjunctive, but propranolol is not, uh, or perhaps straws, but. At least the times I've seen recently, if you have a kid that's in failure, right, they're in heart failure in the ICU, right, then they'll usually get beta blockers again steroids that you're trying to trying to do everything, but I think it, you know, kid would just pick it up so. All right, so we've gone through 3 of these, um, with the third one being I would consider relative. By the way, have, have, have any of us seen? I've never seen a compulsive form hemangio endothelioma. This is 1 or 2, yeah, yeah, as you mentioned, we don't see it because we don't pick it up. Yeah, we don't pick it up. Maybe that's it. Maybe they're there and we just don't hear about. Yeah, but I, I. I felt like when when I was preparing for this, that I was learning about something that I knew very little about. But they they, they, they don't this is, uh, of distinct making sure this that this is very distinct from an I'm not confusing the two because they are two different treatments. Into that that mysteries no I think you must be bigger than resolved. This is kind of. Yeah, and in terms of kind of clarifying the differences here, right, so, so these are rapidly enlarging these patients tend to be fairly sick of hearing they're kind of a big deal, um, and then importantly these patients have essentially consumptive gland, right? So their platelet counts are very low, um, and that's why the, the CBC that you mentioned the checks. And of course they also have uh prolonged um uh graduation practice. So, corticosteroids, increasing interferon. All right, so number 4, question number 4, now we're back into the realm of things that we actually see and we've heard about. Um, so this question for, so this is a, uh, large Uh, cervical facial lesion is noted on a prenatal ultrasound. Um, And it's uh concerning for a lymphatic malformation. Uh, unfortunately, I don't think we have either Steve or Katie. Um, with us here, but, uh, their insight on this question would probably be very helpful. So, The mom seeing you in clinic prenatally, and the, the question is, um, and she's concerned about her baby's ability to breathe after delivery. How would you address that? Um, I think in this case I'd be concerned that it's a cystic fibroma. Um, Certainly if there's concern for airway compromise, uh, Um, you have delivery with EMT available. Um, Based on growth, but if there's, if there's no concern for airway compromise then. The insurance. Um, so we've had a, an ultrasound, um, You get more energy. Um, The only other thing I would consider would be a fetal MRI, um, but you could also follow with serial ultrasounds. Excellent. So, um, the, uh, imaging question resulted in an MRI and, uh, uh, what do you see in there on that? Caramacci, what, what, what do you think you see there on that? Uh, uh, fetal Which is actually fairly old. I might have. The newer MRIs, the imaging quality and tissue brains are a little bit more distinct, but it's an ancient image probably from the early 2000s. Let's see a mass of the. All right, so we're talking about we're here we got a brain. Right. And I facial area, neck. Here's the spine coming down, kind of a lung, heart issue area, abdominal cavity area down here. Maybe even some fingers. Um, and here you're talking about A mass in the neck area, right? OK. All right, with that, How do you want to talk to him? Um, But I think that this is something that will uh follow serially, uh, with ultrasounds. Um, Box profiles and And watch for growth and enlargement. OK. So we, um, a few minutes ago, you're kind of talking about, um, the time of delivery. Um, how would you, how do you manage delivery for this patient? Um, So again I was concerned I could have uh ENT available. Um, But this is, this is, uh, likely something that where a planned delivery would be. Prudence. So you could, you could schedule a C-section with this baby. I turned several weeks. So, um, I is this a, I, I guess the interesting question is, is this a patient with whom you The next procedure, uh, where I, I, yeah, I haven't heard, I have not heard of that. I guess it could be possible, um. But if you're able to control the airway, I don't think you would need emergent control. But I haven't seen that. OK. I, I will freely admit I am not a content expert in terms of um when to and how to escalate the resources at the time of delivery um uh to a certain extent. I probably. Steve Fenton or Katie Russell are probably more knowledgeable in terms of, of how to I think about, um, The resources needed at that time in a in a coherent way. Or do you have any thoughts on that? Uh, we've had a couple of these that we've had and exits, um, do you know what the cardinal. There's no one exit procedure is. Um, so they basically leave the child on single bypass, so the child. the child hooked up to placenta bypasses there's no oxygen in the airway. And the plan for those kids have been to. Innovate them on placental bypass. So It's my experience that these lymphangiomas are all soft. They don't threaten the airway, uh, so I, I think. I don't know if the energy is faded away for these in terms of uh That sort of Logistical Uh, effort because they're not hard, they're soft and too many people breathing your own. OK It's a. Yeah, it's different than a terratosa. So Right, and that's actually kind of the, the critical point I'm sure um you're thinking about is, uh, you know, these, these, these lesions are very soft. Um, and so when, when at the time of delivery, um, as Eric mentioned it is relatively, uh, relatively common to be able to pass an endotracheal tube even if it's, uh, the, the lesion itself is obstructing the airway, um, usually get an endotracheal tube in these babies. Establishing there. The alternative on this is, is the teratoma. The more solid harder region of course and they tend to compress the airway, uh, and, um, they also tend to be a circumstance where it can be very that uh in the rates, um, so. Cystic lesion in the neck. can be a problem, uh, and Let's see here. Um, There may be a, a, a need under some circumstances for um resources at the time of delivery. So assuming that the baby can be born, um, and, uh, in fact the lesion is really soft, the airway is not compressed, the baby can breathe, um, and you don't need to Either you intubate, are able to excavate fairly quickly or there's no need for intubation. Um, if left untreated, um, What complications, uh, most often develop in these, in these patients. So I think of the anatomic issues, and so compression and the bas obviously we're not talking away anymore, but um. OK. Very good. Any other ideas? There you go. So infection, right? The, the, yeah, exactly. So, um. Uh, infection. Uh, and in fact, interestingly enough, um, When I was in training, uh, There, there were several staff that I worked with that kind of liked it when it got infected. You have a sense for why? Yeah kind of for the same reason it's, I mean it's sclerotherapy. It's basically sclerotherapy, right? So once it gets infected, you know, there's inflammation, tissue damage in the area and things kind of start to start up. Um, and so, uh, yeah, obviously infection is never a good thing, um, at, at the time in which the infection occurs, but you get, you get one of these infected, treated appropriately with antibiotics and, uh. The infection actually tends to help them kind of scar down a little bit. Um, so, uh, kind of talking about sclerotherapy, um, What characteristics of lymphatic malformations lead you to consider uh sclerosclerotherapy or resection, right? So, uh, these are bulky lesions, um, they're Potentially, uh, amenable to resection, um, so you can go and surgically remove them. Um, but You mentioned sclerotherapy. When is that test? Well, so, I mean, certainly with a big lesion, um, you know, removing it, reconstruction could be particularly difficult, um, could be disfiguring, um, you make sure you have complete resection. But how important is complete resection? Rarely or never achieve it. Yeah, I was gonna say, I was gonna say I, well, I was thinking based on size most, I mean, I think I've only done a couple of these that have been pretty small like we have one on the elbow. I think, I think you and I had, and we're able to completely remove it. That's a principle where I'd worry about something or head and neck, you're not gonna understand. I think the other big thing is nerve. It is we used to like it and it is for that because you've got all those craniofacial nerves in that they're tiny. And I, I think the real risk of it is, is your 100%. Not, I mean it would be the, yes, right. And even, even those that are say on, on the elbow or for whatever reason chest wall, we seem to see these with some degree of frequency, um, when I am involved in, to be clear, it's been several years now since I resected one of these surgically, um, my conversation preoperatively with the family is always that I, I'm going to go after what I can see and my goal is to essentially make the, uh, remove. The, the mass in terms of its effect. visually, um, but the overwhelming likelihood is is that there will be tentacles of this that I do not know. Um, and oftentimes these tentacles are irrelevant, don't grow. You never worry or see, uh, problems with those, um, but occasionally these do actually go back. Um, and so I, I, uh, I make it very clear, uh, have made it very clear historically. And when I used to do these, um, That, uh, my goal is not necessarily to, like, I do not anticipate completely removal. I've had a work looks like you get really excited and we get little, little bubbles in the scar so I would anticipate that. Yeah, and a lot of times again with, again many things with uh pediatric surgery and it's important to set up prepared expectations um and this is one of the circumstances where it's really important to do that. Um, all right. So what characteristics of lymphatic malformations would lead you to consider sclerotherapy, um, rather than, uh, resection? We just discussed a neck lesion in general, um, uh. Probably not excited about doing any reception. What other, what kind of specific natures of the lymphatic malformation would, would, uh, lead you towards therotherapy? Any ideas? Yeah, maybe some imaging characteristics. Um, Well, they're like, I mean, it's like thousands of cysts, right? OK. But, I don't see that as like over reception. Yeah, you actually, that does, that does actually tend to make a, a pretty big difference. So in terms of um uh these are characterized oftentimes as microcystic, macrocystic and mixed. Um and in terms of just kind of the practical aspects of sclerotherapy, in order to do sclerotherapy, you have to get a needle into Something and push the agent into that. Um, and so if you've got a macrocystic lesion where the, the, the individual cysts within the lesion are fairly large. And that, that, that, that's the perfect lesion for sclerotherapy, right? Because you, the interventional radiologist can find one of these larger uh cysts or many of them inject them, uh, and then they will actually shrink, disappear. The microcystic ones, um, yeah, their ability to access these little tiny microcysts virtually zero. And so a microcystic lesion, you're, you're, that, that's the one that's headed down the path potentially getting surgically resected. Um, in fact, I cannot remember, uh, Surgically removing the necrosis lesion. Any comments on it. Yeah, as a fellow I mean that literally is always waking up to it because it's got normal voice where they can move their face I mean it's yeah. So benign. And it sounds like you, in, in the year you've done 2 weeks. I think I can think of two. That's it. They're just a little subcutaneous, but yeah, I think that is, you get in that whole debate of this. I mean, I think there's a little, I mean that is, you wanna follow it, do nothing. You wanna mess around a square. You want me to cut it out. You're gonna trade it for a scar. I mean, it's, that's right. Each family, yeah, that, that ends up being the conversation, right, is you've got a bump. I can trade that bump for a scar that may turn back into a bump later on, right? Um, and so that's, that's the conversation that I'm having. OK, macrocystic sclerotherapy, macrocystic, that's the one that gets done, um, for a sec, and, and if it's in the head or neck, you call for. All right. Yes, that was 4. All right, fine, this is the last question. Um, so you got a, a child, um, brought to your clinic for a second opinion and this question is directly out of sport. Um, and there's some funny components, components to this question. So I brought to your clinic about a second opinion for a vascular malflammation. Um, he was initially told he has triple, uh, uh, renounne. I don't know how to pronounce that, I guess Trenounne uh Weber syndrome as an infant, um. And his new pediatrician that he has said he has, uh, Clove syndrome. Um, he has a bulky lower extremity, one of them, uh, which makes it difficult for him to find pants and shoes to fit, um, and a surgeon has recommended to him that he get the, uh, extremity, uh, amputated. And I did find a photo um uh online of uh theoretically somebody with it. A larger right leg from the left leg. Ever see the difference there? All right. OK, so, um. What features differentiate clothes from uh uh Clippo Trenounne and Parks-Weber syndromes. Um, and, and specifically, um, I think it's important to note that there's no such thing as a uh Clipple Trenounne-Weber syndrome. Um, that's not a thing. There's a Clippo-Trenounne syndrome and a Parks-Weber syndrome, uh, and of course, uh, close. So what, what features uh differentiate this, and I recognize we're, we're pretty esoteric. Yeah, I, I, I mean. Clothes is a, is a congenital overgrowth syndrome. um, and what what what are the nature of those overgrowths? What, what's, what's in. Um, Uh, I think they're lymphatic. They are lipomas, right? So they're fatty. So yeah, cloves is basically fatty tumors. Um. So I, I think one of the keys here is it's been present since birth. OK, so it's a congenital lesion that's, uh, grown with the patient. Yes, the patient's grow. Um, but I gotta be honest, I don't remember the other two. Yeah, that's fair enough. These are, these are, uh, rare, and I'm sure you've never seen. So yeah, OK, so Specifically here, um, I've just added this information. So, um, the, the key thing to remember about clothes I mentioned is that they're lipomatous overgrowths, um, uh, and they can also have some vascular malformations and some acral musculoskeletal anomalies. Um, as, as the, as I mentioned earlier, the, uh, Clippel Trenounne-Weber, uh, syndrome does not exist. It's not. Um, clipple renounate is a slow flow malformation. These are what are called capillary lymphatico venous malformations and Parks-Weber are fast-flow lesions, um, that are capillary lymphaticical arterial venous lesions. Um, and importantly the Parks-Weber. Lesions can cause a high output cardiac flow. So the, the three different things here, uh, cloves or uh uh lipomatous along with uh vascular and they're oftentimes even musculoskeletal issues involved. Um, in fact, years ago, Um 7 or 8 years ago, probably more, um, before Steve was here. Um, I was Uh, attending and, and spending time with the, um, Um, Uh, maternal fetal medicine folks over at the University of Utah we had a, uh, the developing fetus, um, in which we're pretty convinced it was essentially whole body clothes, um, and had several conversations with, um, I wanna say Ari Fisher, uh, in Boston, um, regarding this patient and eventually decided, um, Excited to have a conversation with the, the parents, um. They elected termination, um, because it was such a, uh, really significant whole body, um. Uh, the, uh, triple trinounic slow flow. So these are the capillary lymphatico venous, and, uh, the partial are fast. OK, so photos here, uh, again, I pulled these off, uh, the internet using Google, uh, quile pronouning again these are vascular, so they're going to have some, uh, discoloration. These are the fast flow so far. Slow, slow and Parks-Weber, uh, again with the help of Google, um, uh, this is a fast flow lesion. Um, I don't know why the arrows pointed down low, but let's pointing something important. Um. So for uh this patient, kind of going back to this patient who, who's been told that they have uh Clippel Treanne-Weber syndrome and uh now a new pediatrician says that he has Clove's syndrome and a surgeon's recommended amputation. um What operations, um, I guess how would you respond to this patient in clinic who's been told that she had an amputation? Um, well, I think diagnosis would be important here cause I, I think with some of the, uh, Uh, like with the slow growth, like physical therapy with compression. Um, could be helpful. I think there's, uh, if there's a venous component, I, I think you could do sclerotherapy or ligation, venous ligation could be helpful. Um, I don't think amputation would be helpful. OK. All right. Yeah, um, there may be some debulking procedures. It seems like we've been involved, and if I know, Doug, you've been involved, Eric, you guys recently, I mean, honestly, I think the answer to this, I would send this kid to a vascular center. No, honest, these are, these are rare. These are rare, and I tell you on the board that's unacceptable. Yeah, yeah, exactly, I feel like we've done a few of these. It, it titled on I did a few but most of gratifying. I don't like anything I'm trying to score. I've seen it. I, I but I don't know that a vascular center here would be able to help you with some of these. I mean, some of these kids have a massive, yeah, yeah, uh, large legs and. Uh, Elizabeth Suka is one of our former partners and trained with sea fisherman Bostro. You know, I did a massive bulk and I think that's kind of what they're referring to. Some of these kids basically taking in skin and skin the leg. And I agree with Doug. It's, it's an operation that Uh, it's not terribly satisfying, and we were fortunate, I think, to massive fluid leaks or anything to the moon, but. I think if I had a really significant one like this, that's it, yeah, that, that's what, that's what I meant by I would say the two fish, um, they're really rare that these clove syndrome kids are um those none of those really look like clothes to me. The clothes. I've seen they have sort of cartoon ones. They have these bizarre they got they've got toes going off and I was trying to see. I thought I had a picture. I had a kid that was a, a Russian orphan that came over and he had basically they. Maybe shoes for him in Russia that look like Mickey Mouse shoes. That were affected, but you know, I don't, I don't think it'd be amputating these kids' legs because they functional and so. Some of the. Yeah, and I'd say we, I saw a bad clothes wise it was in Africa we know about it, and I think the general posture is you even see would be like don't operate. I mean, you really try to palliate them more with compression and so forth. I think at a practical level, I think you remember aboutity is there. So I think that's a, that's a place that if you have a general surgeon that's gonna interact with them that's be aware that, you know, that if they committed that you that you should scan. Yep. All right, so that's, that's the next question, which is, it's OK. And, so yeah, what prophylaxis, yeah, so the, these patients, yeah, they clot, right? So, uh, the critical thing here is that they, they have these large persistent, uh, embryonic veins, um, and so perioperative coagulation is critical, um, and certainly prior to any procedure really. Um, interesting that they actually talked about putting in the cava filter, um. I'm not sure. Probably would not be putting patients at actually at this age, but, uh, that it's such a big deal that's something to consider. So, um, just in a, uh, last few moments before we finish, just a couple of, um, Uh tables from the not a textbook. So when you're thinking about uh vascular tumors. You've essentially got your infantile hemangiomas which we discussed earlier. Propranolol. Corticosteroids, mainly propranolol, uh, sometimes embolization in the liver. And for cutaneous, subcutaneous fruit lesions, right? Your strawberry lesions, um, these tend to resolve spontaneously. They may have, uh, some residual skin component, um, uh. The thing that may be involved, uh, and then you've got these aggressive, um, uh, kaposiform hemangio endothelioma, um, that we generally do not resect, um, but are treated with, uh, uh, vincristine corticosteroids, uh, I can't remember the third one, my, uh, and then of course there are some rare malignant lesions, um, for the vascular malformations, um, we talked about the Uh, capillary malformation, the lymphatic malformations, venous malformations, and then the arterial venous mal malformations. They kind of fit into these, uh, uh, Pipal Trenane, close syndrome, and Parks-rubber syndromes. And that is all I've got. Arterial venous, um, uh, venous malformations, um, uh, some of them are very common. Most of those are really, really Almost clinically irrelevant sometimes, um, and they can range to the spectrum really complex. Um Thank you. The, um, I was looking through the emails for she's apparently. Managing ground grand rounds just this uh informational heads up. She's played it like at Boxdale in the bed be around. We haven't, is he gonna be here physically, I think so. So like I said, you know, how are we gonna engage with him in the great if we want to, yeah, it's great and, and which.
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