Don’t Be Rash: The Pediatric Dermatology Podcast

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Welcome to the Don’t Be Rash Pediatric Dermatology Podcast, the owner’s manual for your kid’s

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skin.

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I’m your host, Dr. K, board certified pediatric dermatologist and father of two boys.

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I’m here to chat with you to promote dermatological education and improve skin health in our children

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everywhere.

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Let’s get started.

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Today’s show is going to be a twofer.

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We’re going to try to cover infantile hemangiomas and port-wine birthmarks.

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Joining me today as co-host and my very special guest is Dr. Caroline Piggott, renowned pediatric

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dermatologist, a mother herself, accomplished figure skater, and one of the coolest people

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I’ve ever had the pleasure of working with and really truly one of my best friends.

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Dr. Piggott did her training in general dermatology with me out at the University of California,

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San Diego.

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We managed to stay together for our clinical fellowship in pediatric dermatology at Rady

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Children’s Hospital, San Diego.

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Dr. Piggott’s clearly smarter than me though, because she stayed there and now lives in

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La Jolla and works at Scripps, one of the most beautiful places on earth.

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Welcome, Dr. Piggott.

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Thanks for joining me from across the country.

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Good morning.

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Thanks so much for having me, Dr. Krakowski.

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It’s good to see you.

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It’s been a long time.

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We’ve been wanting to do this for a while now, so I’m so excited.

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We finally get to put it to the test.

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No, I’m glad to do it.

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So Dr. Piggott, let’s jump right in.

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How do you approach a red birthmark in a child and, ultimately, how do you make that very

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specific diagnosis of infantile hemangioma?

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The babies present to me usually a couple of weeks of age with their parents who are

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usually somewhat-to-very concerned.

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The first thing I do is get a little bit of history.

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So I ask them, was it there at birth or when did you first notice it?

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Because one of the key things about hemangiomas is they present at some point, usually in

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the first month of life.

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Some of them are actually present at birth, but some of them are completely absent at

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birth and present maybe one, two, three, four weeks of life.

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And they often start out flat, like a little red mark.

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Many parents will say to me they thought it was a bruise or they thought they had pinched

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the child or something like that, and then they’ll give me a history that it gradually

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starts to get a little bit thicker and maybe darker.

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Do you see them in any particular anatomic location or they can be all over?

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Oh, they can be anywhere, head to toe really.

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Sometimes parents won’t even notice because it’s in the private parts and they don’t

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really look very carefully or in some babies who are in the hospital, for example, if they’re

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born prematurely, they might be in the NICU.

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Some parents don’t even notice them because they’re covered by leads or diapers or things

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like that at first.

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So they can really be anywhere.

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Yep.

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Up on the scalp.

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If you’re born with a bushy head of hair, you might not notice it.

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Exactly.

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Exactly.

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Or parents will say, we thought it was just from the electrode on our child or something

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like that.

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We’ve been fooled a couple of times with, like you said, ones that are sort of between

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the, for lack of a better word, the butt crack.

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You don’t know what’s there.

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Either the scrotum and a male child, you might, you could find them.

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They can be anywhere really.

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So do you see them always by themselves?

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Is it an isolated thing or can you see these present in different ways?

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So the most common form is when they’re solitary and there’s just one.

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And that’s what I see most commonly.

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But there are actually cases where you can have multiple hemangiomas.

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It’s very important to count how many there are because there’s a rare condition where

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not only do you have hemangiomas on the skin, but also hemangiomas inside the body.

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We call it systemic hemangiomatosis.

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What I always do is, you know, the parents might not even know that there’s other ones.

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So what I always do is completely undress the baby and examine them head to toe.

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And I actually count them.

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The risk of having ones inside your body.

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There’s no clear consensus, but I would say on average if there’s five or more on the

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body, the risk of having one inside the body is a little bit higher.

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And sometimes if there’s five or more, or sometimes even four, because that’s close

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enough, we do actually imaging.

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The most common type of imaging being an ultrasound of the abdomen because the most common area,

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if you’re going to have one inside the body, is actually the liver.

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So an ultrasound is easy to do, no radiation, not harmful for the baby, no sedation needed,

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and you can actually do an ultrasound to make sure there’s not one in the liver.

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And they can see them pretty easily on ultrasound.

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Very easily.

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I mean, when I get the reports back, it’s either there or it’s not.

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It’s very helpful.

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Exactly.

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Exactly.

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And the reason that’s important is because if, let’s say, there was one in the liver,

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if it’s small, it doesn’t really matter, but let’s say there is one in the liver that’s

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large, it could be pushing on the rest of the liver and impact its correct function.

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It could affect circulation.

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And there’s even some, I believe, data about affecting thyroid.

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Yeah, it can definitely impact thyroid function, especially when you have diffuse hemangiomatosis

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on the liver.

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But…

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Again, this is all very rare stuff.

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Most of the time, even with multiple, there’s nothing inside the body.

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Yeah.

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And most of the time, since you brought up the most usual presentation, most of the time

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these are isolated.

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I think one of the hardest parts for pediatricians and family medicine practitioners out there

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and parents is trying to figure out to differentiate between a flat hemangioma, what we might call

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like a superficial one, versus something like a port wine birthmark, which we’re going to

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talk about in the second half of this show.

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But hemangiomas don’t have to be flat when you first see them either.

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Want to talk a little bit about how they might show up from the way they involve the skin?

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So there’s certainly ones that are flat.

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And there are some that are actually raised above the skin.

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And they can be quite significantly raised.

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And those will look very red.

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The reason why we call them “strawberry hemangiomas” is they often look like a strawberry.

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There are also ones that can present under the skin, which makes our job a little bit

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more challenging because when they’re under the skin, they sometimes look blue.

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Just like when we look at our veins, our veins are full of red blood, but under the skin

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they look blue.

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So that can sometimes make the diagnosis a little bit more challenging because there

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are actually other things under the skin that can look blue that would be on the differential.

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And I’ve had some hemangiomas where there’s no color at all.

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You have to get imaging and you find out, oh, it’s a really deep hemangioma.

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You just can’t see the surface of the thing to even know there’s blood in there.

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So you can be fooled.

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Now you tell me how you approach your patients, but my sort of go-to spiel is, listen, just

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like you said, these things usually will follow a stereotypical course if we’re lucky.

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They’re going to maybe not be there at birth.

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They could be there at birth.

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That’s one of the things that helps us differentiate between conditions like congenital hemangiomas,

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which are a little different than infantile hemangiomas, both in what they do, but also

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maybe how they even got there in the first place, which we can talk about if there’s

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time.

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But most of the time there’s really nothing there at birth.

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And then within, like I usually say, about a week or two, you’ll see a cherry red spot

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that then grows pretty quickly.

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And I’ll tell them to expect a lot of growth in the first three months, even up to like

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five months.

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I’ve seen some growth go.

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And then these infantile hemangiomas will sort of transition to what is called the plateau

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phase, where they’re not doing anything.

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They’re just hanging out.

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They’re not getting bigger.

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They’re not getting smaller.

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They’re not causing any trouble.

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And then fingers crossed around, I don’t know, I tell people usually around 10, 11, 12 months

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of age, fingers crossed, nature starts taking over.

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And without doing anything, most of these birthmarks, these infantile hemangiomas, start

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to want to go away on their own.

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And if you’re lucky, I usually quote that about half of them are gone by the time you’re

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five years of age.

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Is that about your spiel as well?

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Yeah.

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I say maybe 20% are gone at age two, 30% age three, et cetera.

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50% are gone at age five.

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Sometimes after that, if at age five it’s still there, there is a chance it could always

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be there.

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Certainly involution can continue up to even seven, eight, nine years of age.

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But some of them actually don’t resolve completely.

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But the vast majority will get flatter, lighter.

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Some completely disappear where you see literally no trace.

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But the other thing that can sometimes happen is when they go through their growth phase,

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they stretch out the tissue a little bit.

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So even though they do get lighter and smaller over time, sometimes the hemangioma may be

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gone.

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But later in life, you see almost like a little pooch of the skin.

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Yeah.

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I caution my patients with hemangiomas that there’s going to be, think of it like a “scaffolding,”

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what someone’s doing, putting a new roof on your home.

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And to get that roof there in the first place, you need to build the scaffolding on the side

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of the house that allows those roofers to get up top there and build their roof.

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In this case, the roof are the blood vessels of the hemangioma.

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And that scaffolding is that connective tissue that then hangs around and leaves, if everything

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else goes away, it could leave behind this sort of residual, I think, or fibro fatty residual,

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I’ve heard it called.

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And that’s a little harder to treat.

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But it still can be treated.

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And that’s where the decision comes as to whether or not you want to treat based on

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the location of the lesion, et cetera.

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Because we do have treatment, of course.

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And that’s where I kind of begin to speak with the parents on, do we want to treat or

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not?

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Yeah.

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So before we get into how we would actively manage these things, optimistically, what

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do you tell patients and their families to look out for signs that these things might

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be going away?

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That they might be getting ready to get ready?

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It’s almost like a grape turning into a raisin.

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Sometimes they start to sort of dry up, shrivel up, get a little flatter, lighter.

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You see almost like a whitish discoloration.

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And so that’s, especially in involution phase, that’s when I tell parents to watch out for

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something called “ulceration,” which is sometimes they dry up, shrink down so much that they

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can even open up.

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Yeah, and also in the rapidly growth phase, if you’ve got one that’s really growing quickly,

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you might see, classically they say there’s like a white streak that you might see as

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an impending sign of doom.

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But yeah, I’ll look for mottling where the color, that red color, a violaceous color

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will turn, darker purple and start to break up a little bit.

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And it gets softer too.

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You can palpably feel it changing under your fingers, usually, that it’s getting softer.

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And man, even within a couple of days, sometimes you’ll see a difference in terms of what this

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thing looks like.

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It’s pretty magical.

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Who’s at risk for getting infantile hemangiomas?

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So any baby could have them, but there is some data in the literature that shows that

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it’s a little bit more likely in a female baby.

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Babies born prematurely.

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And the reason for that, we don’t completely understand, but there’s some, there’s a hypothesis

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that might have something to do with a lack of oxygen compared to full term babies, but

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I would say female and premature babies most common.

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Do they still say twins are more at risk?

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I think so, right?

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Maybe, yeah, multiple gestation.

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And then the question is also, could that be due to prematurity too?

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Right, right, of course.

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Low birth weight, I think is, that’s probably one of the bigger things that I think of.

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But we saw tons of them.

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I mean, that’s for sure.

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We – you and I – trained and practiced in a time where the only treatments really were giving

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it time and letting nature do its thing or putting kids on systemic steroids or even

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worse, something like an anti-cancer medicine in the real bad cases.

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And that’s, we were, I think, I don’t use the word lightly, but we were sort of blessed

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to witness what, at least I tell people was the first miracle I’ve ever seen in medicine,

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you know, the birth of oral propranolol.

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Oh, yes, that was right when we were in residency that they discovered it.

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And I remember before then we’d have babies on prednisone for months, which we know is,

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you know, not an ideal situation.

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I remember even ones where they were close to the eye, they used to have to inject steroids

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into them.

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So it was such a miracle.

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Putting a needle into a kid’s eye and hoping that the medicine you’re injecting

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is going to save their vision was a different experience.

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And we were pretty fortunate.

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We trained at a place where, you know, one of your colleagues there at Scripps, Sheila

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Friedlander, MD – when she worked there – she was absolutely on the cutting edge of what was

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going on in hemangiomas in general.

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And then I think literally you and I were there when we were doing some of the biggest

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research and clinical trials around the medicine that eventually came out to be the “gold standard”

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for treatment, the stuff, oral propranolol.

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And I think it’s so amazing how they figured it out initially, too.

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They were treating babies in a hospital.

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Was it in Spain or something?

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France, Bordeaux, France.

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Who needed propranolol for, I think, cardiac indications.

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And some of them happened to have hemangiomas and they started to notice that they were

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getting better.

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And that’s how it all started.

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To me, that lady who, I mean, granted, she got her respectful dues by getting a publication

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out of New England Journal of Medicine.

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And anybody who knows the story, you know, thinks she walks on water.

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But just if you pull up at 50,000 feet and just understand what it took

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for her to figure this out.

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I mean, like you said, she had a kid on, from what I understand the story to be, was she

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got called for a consult in the neonatal intensive care unit at the hospital that she was at

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for a couple of kids with hemangiomas.

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And just in parallel, they had been started on an oral beta-blocker, or probably actually

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was probably systemic, I would think, because it was the NICU.

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And she was able to then do two things, which I give her way more credit than what I would

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have been able to do.

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A, she pieced this all together.

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And B, she did it because she actually went back very quickly to check on how these kids

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were doing after she started them on oral steroids, and realized that,

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geez, I think this is too soon.

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The effect that we’re seeing is too soon to have been the consequence of these steroids,

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which we know took months and months and months to work.

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And somehow she was able to put that together in her brain.

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And to me, that is like, I don’t know, at least within the world of dermatology, that’s

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Nobel Prize worthy, because she made that connection and then had the guts to look at

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it more formally, write it up, submit it to the New England Journal of Medicine as a brief

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report.

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And now it’s the gold standard.

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And it’s totally changed the way we do things, thankfully.

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And so before we dive into management, because that’s obviously one of the positive sides

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of this discussion, you mentioned a little bit about how hypoxia may play a role.

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Do you remember when you were training what the old thoughts were that this was caused

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by?

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I don’t want to plant that in your head, but I kind of have a weird story in my mind of

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being told what this was and going, oh, that’s interesting, and then just buying it as totally

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ex-cathedra, but then figuring out that down the line that that didn’t make any sense.

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Does that trigger anything?

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No, no.

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So I remember being told that this was probably a chunk of mom’s placenta that got broken

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off and was now being passed through the kid and was quote-unquote “growing.”

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Oh, and that’s what the hemangioma is?

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Oh, yeah.

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I know.

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I never heard that.

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And I was, wow, my goodness, that sounds horrifying.

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And then you’re like, yeah, that’s probably not really it.

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But to your point, a lot of research has been done and this concept that hypoxia, that’s

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the technical term for it, but just lower oxygen levels in the tissue.

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Not that the baby is suffering from any sort of low oxygen state, but the idea that the

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skin itself took a little miniature “hit” in terms of how much oxygen was going through

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it.

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And then could it be compensating by then sort of bursting out with blood vessels that

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try to get more blood to that area because that area particularly was lower in oxygen.

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That to me not only makes sense, but it’s backed up by a little bit of the science.

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So we know these infantile hemangiomas, they’re Glut-1 positive.

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Can you speak at all about how you use Glut-1 as a marker for these things?

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Yeah.

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Glut-1 is “glucose transporter 1.”

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And one of the things we actually do in the clinic, for example, let’s say we don’t know

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if it really is a hemangioma or it’s like one of those subcutaneous ones and we’re not

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sure if it is one and that determines how we treat it.

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If you actually take a biopsy of a hemangioma, you can actually stain it for that and that

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can help you determine if it’s a hemangioma versus some other sort of venous malformation

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or whatever.

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And if the biopsy is Glut-1 positive, you then know it is a hemangioma.

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It’s super specific to these particular things, right?

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And it can be helpful too because there’s some rare variants of hemangioma, not really

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a variant but an alternative type of vascular lesion.

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There’s one called a congenital-type hemangioma and there’s actually two different ones,

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a non-involuting congenital hemangioma and a rapidly involuting congenital hemangioma that

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look very similar but have different courses and one of the things we do to differentiate

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a hemangioma of infancy from them is actually do the staining on a biopsy.

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And, God forbid, there are a couple scarier “blue things” that can start to grow in kids

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that we know about, tufted angiomas and let’s see if I can say this one correctly.

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The Kaposiform hemangioendothelioma.

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Hachoo!

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It’s a mouthful.

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But those two are famous because they can cause a devastating problem called Kasabach-

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Merritt Syndrome where platelets get kind of stuck inside the lesion.

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Totally something that we don’t see in infantile hemangiomas but sometimes you don’t know

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and biopsying can make the difference for you.

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But that said, when’s the last time you biopsied a hemangioma?

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Not often because hemangiomas are made of blood vessels and when you’re in your outpatient

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clinic without an OR you have the risk of bleeding.

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So not my favorite thing to do.

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Yeah I think we did it a couple times maybe during training but at least in the last five

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years I have not stuck anything into a hemangioma.

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Rather than I guess stuck on might be a way to phrase it, I think we’re in a time now

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where some of the management options that we have do afford us a week, a two weeks period

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where we can actually say hey let’s try these medicines that really should only work on

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infantile hemangiomas.

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00:19:58,360 –> 00:20:04,800
It would be very helpful I think to have almost automatically when you’re asking that ultrasound

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a hemangioma would be great if the radiologist automatically did the flow.

333
00:20:09,480 –> 00:20:11,680
Sometimes you have to go back and ask for that to be done.

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I don’t know about your institution but.

335
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We do have to ask.

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One of the things I wish we had been trained to do it ourselves even.

337
00:20:18,080 –> 00:20:19,680
Oh that would be great right?

338
00:20:19,680 –> 00:20:23,600
Right at the bedside would save a lot of people a lot of worries but ultimately we get the

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00:20:23,600 –> 00:20:29,320
answer and it is very reassuring when you see that it’s a “fast flow” lesion

340
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not “pulsatile” like you said in arteriovenous malformation in AVM. Not a slow flow lesion

341
00:20:35,160 –> 00:20:39,320
like a venous malformation but the blood going through these tiny little capillaries that

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00:20:39,320 –> 00:20:42,720
make up this what really is a tumor of blood vessels, right?

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00:20:42,720 –> 00:20:47,520
That’s a big thing that and a scary thing to hear for parents but when you’re kind of

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looking at red birthmarks on a kid the way at least I kind of characterize them and make

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the first split…is this a tumor of blood vessels or is this a malformation of some vasculature

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and hemangiomas fall into what is technically a bucket of tumors in the sense that it’s

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not a cancer it’s not going to spread and take over the person’s body but it’s a tumor

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it’s not supposed to be there and it’s living growing tissue that has sort of connected

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itself in a place that’s not supposed to be.

350
00:21:21,440 –> 00:21:28,040
Speaking of places where it’s not supposed to be what anatomic locations get you nervous?

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Well the two main ones would be face with that having both medical and cosmetic implications

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and then the second one would be the genital area and there’s a couple of reasons let’s

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start with the face so I always tell my patients there’s two issues medical and cosmetic so

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medically on the face especially in places for example can you imagine on the eyelid

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where a hemangioma might grow and push on the eye or impede the baby’s ability to open

356
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their eye that can affect vision.

357
00:22:02,960 –> 00:22:08,600
On the nose…imagine, you know, it being near the airway. It can affect a baby’s ability

358
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to breathe. And then certainly on the mouth…the baby’s ability to feed and

359
00:22:13,400 –> 00:22:18,640
then another consideration would be the size of the hemangioma because there’s this rare

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condition we call it PHACE syndrome where you have a large hemangioma commonly kind

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00:22:24,120 –> 00:22:30,640
of in this distribution of the “beard” area there’s a condition called PHACES syndrome.

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00:22:30,640 –> 00:22:40,440
P-H-A-C-E stands for the P is for posterior fossa malformations

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00:22:40,440 –> 00:22:48,000
The H is for hemangioma. The A is for arterial anomalies. The C is for cardiac defects or

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00:22:48,000 –> 00:22:56,000
also actually aortic coarctation, and the E is for eye anomalies and the most common location

365
00:22:56,000 –> 00:23:01,560
for this syndrome is to have a hemangioma on the face so I don’t know what your experience

366
00:23:01,560 –> 00:23:02,880
with those has been.

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00:23:02,880 –> 00:23:07,180
We’ve had a couple over the last five years that we’ve had St. Luke’s Dermatology up

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and running, and they they’ve ranged from being caught early on and being effectively

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managed now with the oral propranol that we have to having some kids that have had consequences

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where you know it would have been impossible to prevent but that they’ve had some of those

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00:23:27,720 –> 00:23:32,600
other findings that you mentioned. More specifically, the the heart stuff is seemingly

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what we tend to find, not – thank God! – not a lot of brain issues or but the but they’ll

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have some heart associated defect and you kind of just lump that in together and say

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00:23:44,400 –> 00:23:48,360
this would be consistent with PHACE syndrome even if it’s totally unrelated

375
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because we don’t have a way to test both directly and say oh yeah this is the this is because

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of the same thing.

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00:23:54,640 –> 00:23:58,560
And not only for the facial hemangioma is the medical side, which is clearly the most

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00:23:58,560 –> 00:24:03,120
important there’s the cosmetic side too. I mean you have this large tumor growing on

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your beautiful child’s face.

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It can distort the tissue we know that they involute in the future but you might be you

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know left with stretched out tissue right in the middle of your face so and which is

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very concerning.

383
00:24:18,000 –> 00:24:24,640
Yeah so I kind of approach it just exactly like you have…is this hemangioma

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going to pose a functional risk and will it pose a long term cosmetic risk. And I think

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00:24:31,960 –> 00:24:38,040
you hit it on the head… Eyes. So we have a wonderful pediatric ophthalmologist that we work with

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that gets these kids in very quickly, makes sure that the optic nerve is intact that there’s

387
00:24:45,720 –> 00:24:51,960
no findings of PHACE. If there is one that’s growing close to the eyelid margin you could

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00:24:51,960 –> 00:24:56,720
imagine if you’re catching that early enough that you’re going to maybe see some rapid

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growth over the next few weeks. This thing could grow literally up and into the field

390
00:25:01,640 –> 00:25:07,960
of vision so – it’s really interesting! – the ophthalmologist will have – and I only learned this by training

391
00:25:07,960 –> 00:25:11,280
in pediatric dermatology but for the people out there who never would have thought this

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00:25:11,280 –> 00:25:19,360
through you actually in those cases – you will purposefully have the child block the unaffected

393
00:25:19,360 –> 00:25:25,960
eye, the eye where the hemangioma is NOT. And that forces the kid to use the vision of the

394
00:25:25,960 –> 00:25:31,040
eye that is being somewhat interrupted by the hemangioma and that keeps those optic

395
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nerve pathways intact and alive, because in pediatrics there is this true phenomenon of

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00:25:37,240 –> 00:25:41,480
“Use it or lose it!” Right? So you want to keep both of those eyes working

397
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equally and it’s kind of cool when you get those kids in and you can make a

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00:25:46,600 –> 00:25:51,800
real difference for them long term. Nose, for sure. we’ve involved pediatric Ears, Nose and

399
00:25:51,800 –> 00:25:58,160
Throat (ENT) in a bunch of cases and probably even more so for the “beard” area. Yeah, how about

400
00:25:58,160 –> 00:26:02,040
the “beard” area? You want to chat a little bit about that? It can actually push on your airway

401
00:26:02,040 –> 00:26:08,400
or esophagus so and there’s some cases where the hemangioma itself might clinically appear

402
00:26:08,400 –> 00:26:13,440
from the outside is completely flat but actually has a deeper component. I remember a case that

403
00:26:13,440 –> 00:26:19,680
in residency where a what we thought was previously just a flat hemangioma the child

404
00:26:19,680 –> 00:26:26,160
presented to the ER with stridor and after imaging and scoping by ENT it turns out it actually

405
00:26:26,160 –> 00:26:31,240
was pushing much below and of course we could fix that with propranolol but it was

406
00:26:31,240 –> 00:26:38,120
quite scary. Yes, what’s stridor for the audience? It’s a funny sound that babies will

407
00:26:38,120 –> 00:26:46,840
will make almost like a…Can you replicate it? Yeah, yeah almost like…GASP. Maybe, is that

408
00:26:46,840 –> 00:26:52,080
pretty good stridor? Yeah I don’t know. Yeah it’s just almost like gasping and and it’s

409
00:26:52,080 –> 00:26:58,560
frightening because the the hemangioma can grow really quickly and actually require the

410
00:26:58,560 –> 00:27:04,080
baby to be intubated if not treated quickly. Yeah so for anyone listening out there

411
00:27:04,080 –> 00:27:08,480
if you happen to have an infantile hemangioma on your child in the sort of the

412
00:27:08,480 –> 00:27:13,520
“beard” area where you might be able to grow a beard that’s a clue for

413
00:27:13,520 –> 00:27:20,640
a kid – a baby. You can’t put them on a treadmill and do a “stress test” so for the

414
00:27:20,640 –> 00:27:25,840
kid that stress test is usually eating. They’re chugging away on the bottle or the breast

415
00:27:25,840 –> 00:27:30,600
and they’re really using all of their energy and so if you’ve got one

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00:27:30,600 –> 00:27:36,200
of those birthmarks in that area and you’re hearing your child make a sound that suggests

417
00:27:36,200 –> 00:27:40,600
he or she’s having trouble breathing that can be a real big sign that you need

418
00:27:40,600 –> 00:27:44,300
to get in there and see somebody and not be told that you’ll get into the dermatologist

419
00:27:44,300 –> 00:27:48,400
nine months from now. You got to just show up at the door and make sure someone sees

420
00:27:48,400 –> 00:27:54,380
you pretty quickly. One other area that I forgot to mention, too, is the ear. What could seem

421
00:27:54,380 –> 00:27:58,540
like a superficial hemangioma can actually have a deeper component and affect the development

422
00:27:58,540 –> 00:28:04,240
of your ear or hearing of your child. So cosmetically I’ve actually had two patients now where if

423
00:28:04,240 –> 00:28:09,240
the top of the ear for lack of a better anatomical description is involved that the

424
00:28:09,240 –> 00:28:14,560
ear itself sometimes there’ll be a segmental hemangioma that’s part of that ear but

425
00:28:14,560 –> 00:28:20,560
then also kind of spills onto the scalp and in those particular cases it really did deform

426
00:28:20,560 –> 00:28:26,320
the top of the ear. In one child that sort of ulcerated and left the child with almost

427
00:28:26,320 –> 00:28:32,720
like a bite out of the top of her ear, and the other child was left with sort of a bent

428
00:28:32,720 –> 00:28:38,720
ear lobe as a result of that so that’s a really important both functional and cosmetic area

429
00:28:38,720 –> 00:28:44,040
for sure. How about in the diaper area? Where do you get into trouble with hemangiomas? So

430
00:28:44,040 –> 00:28:50,520
same issue medical first most important depending on how big it is or where it’s located it

431
00:28:50,520 –> 00:28:58,840
can affect a baby’s ability to urinate or stool. I’ve had a baby where

432
00:28:58,840 –> 00:29:03,040
it completely impacted the perianal area and we had to do propranolol so the baby could

433
00:29:03,040 –> 00:29:08,720
even have a bowel movement. You know it was blocking their ability to

434
00:29:08,720 –> 00:29:16,320
poop. Yes okay yes and there’s also a rare condition especially with a very large hemangioma

435
00:29:16,320 –> 00:29:22,000
we call it a segmental hemangioma. There’s sort of the the opposite of PHACE syndrome

436
00:29:22,000 –> 00:29:30,520
in the area we call it LUMBAR syndrome: L U M B A R. Basically it’s a lower body hemangioma

437
00:29:30,520 –> 00:29:40,000
is the L. The U stands for urogenital anomalies and ulceration. The M stands for myelopathy.

438
00:29:40,000 –> 00:29:48,240
The B stands for bony deformity excuse me. The A stands for anorectal malformations

439
00:29:48,240 –> 00:29:55,120
of which there can be multiple and also arterial anomalies, and then the R stands for renal

440
00:29:55,120 –> 00:30:01,640
anomalies.. And so I actually had one in clinic in the last year who presented with a large hemangioma

441
00:30:01,640 –> 00:30:08,160
basically from the top of the labia all the way to the buttocks on both sides had to be

442
00:30:08,160 –> 00:30:16,600
worked up for this. And what we do is some MRI imaging to check not only the urogenital

443
00:30:16,600 –> 00:30:22,760
area also the lower spine and fortunately this baby turned out to be totally fine. It

444
00:30:22,760 –> 00:30:27,840
was an isolated hemangioma but you have to look for these other anomalies. Yeah that

445
00:30:27,840 –> 00:30:34,360
must have been horrifying. Thankfully that’s pretty rare correct? And and so

446
00:30:34,360 –> 00:30:38,800
once you rule that out the main issue in that area is then because you know you can imagine

447
00:30:38,800 –> 00:30:44,880
having a large growth in your diaper what’s the main issue that happens? You have you know

448
00:30:44,880 –> 00:30:50,120
stool, urine in the diaper and you can get actually erosions, ulcerations which present

449
00:30:50,120 –> 00:30:55,480
not only in a very painful way but also at a high risk for infection and bleeding. My

450
00:30:55,480 –> 00:31:01,040
spiel for that is, you know, if you think about it urine’s got ammonia in it and all the digestive

451
00:31:01,040 –> 00:31:08,360
enzymes that start from your mouth all the way down to your anus winds up getting dumped

452
00:31:08,360 –> 00:31:13,840
into the diaper. These diapers are right up against the skin. Even without a hemangioma

453
00:31:13,840 –> 00:31:19,880
you can get “diaper dermatitis” a diaper rash where the real underlying cause is simply

454
00:31:19,880 –> 00:31:25,480
just an irritation from those materials up against the skin. Now you’re putting those

455
00:31:25,480 –> 00:31:32,840
same caustic chemicals onto not normal skin but this infantile hemangioma skin – segmental

456
00:31:32,840 –> 00:31:38,120
or otherwise. It can just be a normal little hemangioma and, man, I find that that

457
00:31:38,120 –> 00:31:43,760
skin is particularly friable and will ulcerate a little bit more easily. So what happened

458
00:31:43,760 –> 00:31:46,880
when we’re talking about ulcerating…You want to explain what sort of that phenomenon is?

459
00:31:46,880 –> 00:31:52,360
What do we mean when we say a hemangioma ulcerates? So for example in the growth phase as they

460
00:31:52,360 –> 00:31:58,280
get larger sometimes the superficial portion of the lesion actually almost looks

461
00:31:58,280 –> 00:32:03,200
like a small tear of the skin as it stretches out

462
00:32:03,200 –> 00:32:07,080
and then it’ll gradually open up and make a sore that can actually get wider and deeper

463
00:32:07,080 –> 00:32:12,200
over time as the hemangioma grows and you can imagine having a sore rubbing against

464
00:32:12,200 –> 00:32:19,000
the diaper. It’s very painful one of the things I think parents should hear is that yes this

465
00:32:19,000 –> 00:32:24,360
is for lack of a better description a big tumor full of blood but when when we get an

466
00:32:24,360 –> 00:32:31,080
ulceration when the skin breaks down it’s not going to show up as a bursting water balloon

467
00:32:31,080 –> 00:32:35,300
of blood. That’s what all the parents think is going to happen. Yeah sometimes they miss

468
00:32:35,300 –> 00:32:40,760
it. They miss the fact that the skin that’s overlying these things is getting eroded and

469
00:32:40,760 –> 00:32:46,980
chewed up and is basically like you skin your leg. You can look for a surface change

470
00:32:46,980 –> 00:32:53,160
that’s really what we’re talking about and, man, it hurts when you have

471
00:32:53,160 –> 00:32:58,180
any break in your skin. A paper cut even feels not great to an adult. Imagine what this would

472
00:32:58,180 –> 00:33:03,400
feel like in your diaper area for a little baby. On top of that like you said any of those

473
00:33:03,400 –> 00:33:07,760
microbes especially in the diaper area from the poop are getting now into the skin when

474
00:33:07,760 –> 00:33:12,160
it normally shouldn’t. Anything can get in there and cause an infection which then can

475
00:33:12,160 –> 00:33:18,280
really exacerbate all those problems so we we take it very very seriously and act very

476
00:33:18,280 –> 00:33:24,500
aggressively when an ulcerated hemangioma presents. What’s your sort of… what do you do

477
00:33:24,500 –> 00:33:29,960
extra in terms of management for those kinds of things? You mean apart from using things

478
00:33:29,960 –> 00:33:34,440
like propranol? Yeah yeah. Well, what else would you do for those? So I tell the

479
00:33:34,440 –> 00:33:40,760
parents how important it is to keep the area well hydrated. We have various different brands

480
00:33:40,760 –> 00:33:46,040
of petrolatum-based products that you can use in the area so I I counsel with every

481
00:33:46,040 –> 00:33:51,400
diaper change you may want to put a layer on top as well as a barrier cream something

482
00:33:51,400 –> 00:33:56,480
for example with zinc oxide to protect the hemangioma from rubbing against the diaper.

483
00:33:56,480 –> 00:34:03,720
That’s great. We’ve even had to prescribe topical lidocaine at times, and

484
00:34:03,720 –> 00:34:08,120
we use metronidazole a lot for when it’s in the diaper area just to try to control some

485
00:34:08,120 –> 00:34:13,560
of the poop associated bacteria. I don’t know if that’s something you guys do but same thing.

486
00:34:13,560 –> 00:34:18,880
But since we’ve been beaten around the bush here now what would your “go to” be

487
00:34:18,880 –> 00:34:25,000
to treat any hemangioma where it’s functionally at risk for causing problems or cosmetically

488
00:34:25,000 –> 00:34:30,680
at risk for causing problems? What’s your sort of management approach? So what I

489
00:34:30,680 –> 00:34:35,700
do is I introduce the family to the concept of there being both an oral and a topical

490
00:34:35,700 –> 00:34:42,840
beta-blocker available. The oral – the most common one we use is propranolol – and the topical being

491
00:34:42,840 –> 00:34:50,880
timolol and what I present it as is an opportunity to shrink the hemangioma down in size and

492
00:34:50,880 –> 00:34:58,200
it also improves the color but also helps lower the risk of development of these ulcerations

493
00:34:58,200 –> 00:35:03,280
But the most important thing I talk about with families is that before starting anything

494
00:35:03,280 –> 00:35:08,040
like this the decision has to be made as to whether you want to do the oral versus the

495
00:35:08,040 –> 00:35:13,480
topical based on the risk and with those risks being the most important ones being the risk

496
00:35:13,480 –> 00:35:19,560
of lowering your baby’s blood pressure pulse and blood sugar and you and

497
00:35:19,560 –> 00:35:23,640
the problem with that is these are babies and not adults an adult’s blood pressure is

498
00:35:23,640 –> 00:35:30,760
you know 120 over 80 a baby’s blood pressure might be 80 over 50 at baseline and the problem

499
00:35:30,760 –> 00:35:35,240
with lowering a blood pressure is then you don’t get enough blood profusion to vital

500
00:35:35,240 –> 00:35:40,960
organs like your brain so you have to be very careful with these medications. We don’t just

501
00:35:40,960 –> 00:35:47,040
give them to every baby. There there especially in smaller ones there is opportunity to start

502
00:35:47,040 –> 00:35:51,840
with for example a topical version of this which is the timolol which has a lower risk

503
00:35:51,840 –> 00:35:57,880
of those side effects although not zero and to a couple more side effects that are very

504
00:35:57,880 –> 00:36:06,360
rare if a baby has asthma you could increase the baby’s risk of wheezing if the baby. So

505
00:36:06,360 –> 00:36:11,720
there’s this rare thing we call night terrors that you can get with beta-blockers which

506
00:36:11,720 –> 00:36:17,600
is basically in a nutshell the baby wakes up from sleep screams shouts falls back asleep

507
00:36:17,600 –> 00:36:22,440
right away, which is very hard to differentiate from normal baby behavior at that age anyway.

508
00:36:22,440 –> 00:36:25,720
But I always tell parents if it seems like it’s happening more often it could be the

509
00:36:25,720 –> 00:36:31,560
medicine and then I’ve also had a couple rare GI side effects there are reports of

510
00:36:31,560 –> 00:36:35,760
constipation but I’ve also had actually some have the opposite effect. I don’t know what

511
00:36:35,760 –> 00:36:41,240
your experience is absolutely and and so reflux as well the baby’s like spinning up a little

512
00:36:41,240 –> 00:36:49,320
bit more and also vaso vasoactive changes so that like where their hands look cold. You

513
00:36:49,320 –> 00:36:53,400
mean yeah yeah look like they went into a cold room and everything turned blue for a

514
00:36:53,400 –> 00:36:57,120
couple seconds and then which is scary for a mother or father yeah it would look like

515
00:36:57,120 –> 00:37:02,480
your kids suffocating you know it through his hands or feet but in really in real life

516
00:37:02,480 –> 00:37:07,400
there’s no known consequence when that happens it’s just like a thing that’s known to happen

517
00:37:07,400 –> 00:37:12,960
with these medicines and I think you hit on a great point so these are blood – this propranolol

518
00:37:12,960 –> 00:37:19,760
and so oral propranolol and topical timolol…topical by the way when we say topical what

519
00:37:19,760 –> 00:37:24,120
we’re talking about is you do not give this to your baby’s mouth. You would be putting

520
00:37:24,120 –> 00:37:30,960
it directly on the skin and in fact just as a little interesting side note from what I’ve

521
00:37:30,960 –> 00:37:36,020
learned of topical timolol…It cannot be given – should never be given – orally because it’s

522
00:37:36,020 –> 00:37:43,360
actually much stronger in terms of its potency than oral propranolol and for that

523
00:37:43,360 –> 00:37:48,080
reason I always tell my families who are using the topical you want to be very

524
00:37:48,080 –> 00:37:54,240
certain who’s giving this medicine – not a child that’s helping take care of the his baby brother

525
00:37:54,240 –> 00:38:01,300
or sister, not a mother-in-law who’s visiting and forgets that this is not to be delivered

526
00:38:01,300 –> 00:38:05,080
into the baby’s mouth with the little dropper. This is going on the skin. That’s what we’re

527
00:38:05,080 –> 00:38:10,160
talking about topical. It’s actually an eye drop as its original function was for glaucoma.

528
00:38:10,160 –> 00:38:16,080
Right yeah. Absolutely. It lowers the blood pressure in a condition called glaucoma and

529
00:38:16,080 –> 00:38:21,920
that’s the point so these are blood pressure lowering medicines being used in

530
00:38:21,920 –> 00:38:28,280
a population of kids with infantile hemangiomas who don’t really normally have high blood

531
00:38:28,280 –> 00:38:33,720
pressure so all the side effects are directly related to taking this anti high blood pressure

532
00:38:33,720 –> 00:38:38,560
medicine when you don’t need those medicines. So like you said lowering your blood pressure

533
00:38:38,560 –> 00:38:44,020
that’s the probably the biggest risk you can cause someone to to to drop their blood pressure

534
00:38:44,020 –> 00:38:49,720
dangerously low. You can die from that. You can drop their blood sugar levels dangerously

535
00:38:49,720 –> 00:38:51,160
low. You can die from that.

536
00:38:51,160 –> 00:38:53,160
You can get a seizure too.

537
00:38:53,160 –> 00:38:59,040
Seizures yeah so it’s not to be taken lightly. But at the end of the

538
00:38:59,040 –> 00:39:03,080
day and I used to have it when we actually I don’t know if you remember but when this

539
00:39:03,080 –> 00:39:08,760
protocol first came out when we were fellows we had to call consults on the Cardiology

540
00:39:08,760 –> 00:39:13,760
team – the Pediatric Cardiology team – every patient that we wanted to start this medicine on and

541
00:39:13,760 –> 00:39:19,720
I had for a while I had it with me I think when I left Rrady Children’s – I think it died

542
00:39:19,720 –> 00:39:25,560
in the ethos – but I had a letter from one of the cardiologists there that said, “Please, please…

543
00:39:25,560 –> 00:39:32,920
for the love of God stop calling me. The doses that you guys are maxing out at are not

544
00:39:32,920 –> 00:39:38,760
even the doses that we usually start our kids on when they do have high blood pressure!” So

545
00:39:38,760 –> 00:39:43,280
probably we were making a bigger deal of it but truthfully that was not something that

546
00:39:43,280 –> 00:39:50,480
we were experts at back then. Now, thankfully, we’ve got what 12, 13 years of experience using

547
00:39:50,480 –> 00:39:57,040
this medicine and I actually heard for the first time in our area a pediatrician was

548
00:39:57,040 –> 00:40:02,760
managing this himself or herself without a dermatologist so it’s sort of it’s

549
00:40:02,760 –> 00:40:07,080
starting to trickle down and I think people are less afraid and realize like with some

550
00:40:07,080 –> 00:40:13,640
good prep time and preparation for the families you can avoid 98% of these

551
00:40:13,640 –> 00:40:18,360
issues. Some are going to happen no matter what but the hypoglycemia…what do you

552
00:40:18,360 –> 00:40:22,360
tell your patients to do to avoid hypoglycemia? You must always give

553
00:40:22,360 –> 00:40:29,200
the medicine with a feed so what I do is I have the mom maybe give a half feed then give

554
00:40:29,200 –> 00:40:32,420
the medicine then finish and the reason I don’t do a full feed is sometimes they’re

555
00:40:32,420 –> 00:40:37,920
so full they don’t even want to take the medicine. And then the other thing I caution is if a

556
00:40:37,920 –> 00:40:43,560
baby is ill like let’s say they have the flu – they’re vomiting or they’re not stooling normally,

557
00:40:43,560 –> 00:40:48,160
or just not feeling good for whatever reason – I have the family skip the dose that

558
00:40:48,160 –> 00:40:54,000
day. There’s no harm to the hemangioma and taking a break for a day or two and it’s not

559
00:40:54,000 –> 00:41:00,840
worth the risk. Very similarly I always counsel the families like look – let’s say little Mary

560
00:41:00,840 –> 00:41:05,680
or a little Johnny is sitting there. You’re getting ready to dose otherwise looks totally

561
00:41:05,680 –> 00:41:12,320
healthy. You give the dose of propranolol and we almost exclusively use and it’s one

562
00:41:12,320 –> 00:41:18,880
of the only times we actually do almost exclusively use the brand name oral Hemangeol, which is

563
00:41:18,880 –> 00:41:25,940
a very specifically studied form of generic propranolol. It’s the same medicine but it

564
00:41:25,940 –> 00:41:32,240
comes in a “twice a day” formulation rather than a three times a day. There’s no alcohol,

565
00:41:32,240 –> 00:41:38,200
which I think is a big plus for dosing babies, and it’s flavored. It’s supposed to be strawberry

566
00:41:38,200 –> 00:41:43,160
vanilla. I never tried it myself but it’s pink and my point here is when you give this to

567
00:41:43,160 –> 00:41:48,060
the baby little Johnny, little Mary sitting there you give them the two mLs or whatever

568
00:41:48,060 –> 00:41:52,760
their dose is supposed to be…They look at you. They smile and they puke it right back up

569
00:41:52,760 –> 00:41:58,680
onto the floor – what’s clearly six mLs, three times as much! What do you do? and I’ll

570
00:41:58,680 –> 00:42:04,640
just pause and I just say what would you guys do and usually someone in the room says do

571
00:42:04,640 –> 00:42:10,520
nothing. That’s the right answer! Don’t give the dose again. Don’t double up on it. You assume

572
00:42:10,520 –> 00:42:15,440
little Johnny, little Mary got the entire dose into their brains and you’re not going to

573
00:42:15,440 –> 00:42:19,960
risk giving them a second dose right then and there. Just wait. If you miss one dose – to

574
00:42:19,960 –> 00:42:24,600
your point, Dr. Piggott – who cares? It’s not a matter of life and death. And

575
00:42:24,600 –> 00:42:32,800
I also – one of the other things – especially in families with multiple caregivers I I remind

576
00:42:32,800 –> 00:42:39,440
them how easy it is to forget that maybe dad gave it in the morning and mom doesn’t realize

577
00:42:39,440 –> 00:42:43,920
it and gives another dose, so I I encourage them to have like a little calendar on the

578
00:42:43,920 –> 00:42:48,480
wall where it’s checked off so you know that the dose was given or I usually or I’ll say

579
00:42:48,480 –> 00:42:53,280
you have just one person be in charge of giving this dose. That’s brilliant! Yeah I like that

580
00:42:53,280 –> 00:42:57,760
a lot. That has happened. Usually for these patients I’m giving them my personal cell

581
00:42:57,760 –> 00:43:01,840
phones letting them know hey if you have any questions especially in the beginning but

582
00:43:01,840 –> 00:43:06,800
two or three months in they’re experts. But there are – and I did one Saturday get a call

583
00:43:06,800 –> 00:43:12,080
from mom that dad had doubled the dose by not remembering that mom had done it so

584
00:43:12,080 –> 00:43:17,440
it’s kind of scary but at the end of the day baby’s fine, healthy, eating, give him a couple extra

585
00:43:17,440 –> 00:43:22,800
rounds of milk and don’t give the next dose and you’re fine. So the other thing to

586
00:43:22,800 –> 00:43:27,520
remember is we’re not starting this medicine “cold turkey,” right? We’re not going right up

587
00:43:27,520 –> 00:43:32,200
to a dose that we would need to see clinical effect on these hemangiomas at least

588
00:43:32,200 –> 00:43:36,120
we’re not I don’t know if you’re still doing the ramp up but…Absolutely! You are? So good.

589
00:43:36,120 –> 00:43:42,680
So the first week or so is a test dose that’s not even supposed to do anything to the hemangiomas.

590
00:43:42,680 –> 00:43:47,640
It’s just supposed to be there to see how the kid’s reacting to the medicine and then

591
00:43:47,640 –> 00:43:52,600
once you establish that the heart rate and the blood pressure are still within healthy

592
00:43:52,600 –> 00:43:58,040
limits then you actually give the second dose which you could expect to see some clinical

593
00:43:58,040 –> 00:44:03,280
effect. If you don’t there’s even a third level which I sometimes don’t even go up to

594
00:44:03,280 –> 00:44:06,640
if I’m getting good effect at the second level I don’t even go up to the third level. How

595
00:44:06,640 –> 00:44:13,680
about you? Agreed and what we sometimes do if the baby is especially in let’s say it’s

596
00:44:13,680 –> 00:44:20,400
a newborn like two weeks old rapidly growing you know PHACE syndrome type baby after we’ve

597
00:44:20,400 –> 00:44:24,440
done the workup we there are cases where we even send them to the hospital to be

598
00:44:24,440 –> 00:44:29,760
admitted for monitoring when they’re started on the Hemangeol or the generic

599
00:44:29,760 –> 00:44:36,080
propranol. Yeah and you know you and I were were just featured in that Practical Dermatology

600
00:44:36,080 –> 00:44:40,120
roundtable that we got to do together, which is kind of cool but we talked a little bit

601
00:44:40,120 –> 00:44:44,080
about how long these kids are on propranolol – when you start to take them off. What’s

602
00:44:44,080 –> 00:44:51,960
your approach there? Well it totally depends on on the case. What I try to do clinically

603
00:44:51,960 –> 00:45:00,280
is when I see that the hemangioma is starting to involute on its own I might either leave

604
00:45:00,280 –> 00:45:05,440
the baby at the same mLs and not adjust the dose for weight gain or sometimes I even go

605
00:45:05,440 –> 00:45:11,560
quicker. There’s actually an article published in the last year or two that suggested approximately

606
00:45:11,560 –> 00:45:17,360
thirteen months or so is when a lot of people start to taper but I actually think I’ve done

607
00:45:17,360 –> 00:45:23,120
it younger than that. Okay I tend to maybe even on the other side of things push a little

608
00:45:23,120 –> 00:45:27,760
closer to a year year and a half especially depending on what anatomical area it’s at

609
00:45:27,760 –> 00:45:33,120
but I think for the purposes of this discussion – if anybody’s out there and wondering if A…

610
00:45:33,120 –> 00:45:38,200
their child has an infantile hemangioma and B are they at risk for any of these problems

611
00:45:38,200 –> 00:45:44,640
the the real key point of this discussion is don’t allow yourself or your family to be

612
00:45:44,640 –> 00:45:49,600
told you’ll get in to see the specialist in six months, nine months. Literally you know

613
00:45:49,600 –> 00:45:53,800
if you have to you insist with the pediatrician, you insist with your family medicine doctor,

614
00:45:53,800 –> 00:45:58,440
your primary care doctor whoever that may be…You say, “Listen I I need to get this child

615
00:45:58,440 –> 00:46:03,000
in to make sure this is okay!” and then you reach out to the dermatologist or if you’re

616
00:46:03,000 –> 00:46:08,120
lucky enough to have a board certified pediatric dermatologist. There’s not a lot of us out

617
00:46:08,120 –> 00:46:13,560
there but they’re enough that most of the time we are tuned in and our staffs tuned

618
00:46:13,560 –> 00:46:18,080
in to know, hey, this could be a real emergency and we make every effort to get these kids

619
00:46:18,080 –> 00:46:23,280
in and be seen quickly. So, with that I think for the last portion of the show we’ll switch

620
00:46:23,280 –> 00:46:28,240
gears just a little bit. We’re still talking about red birthmarks but I want to

621
00:46:28,240 –> 00:46:34,440
focus, Dr. Piggott, on port wine birthmarks. We know these things also go by a couple of

622
00:46:34,440 –> 00:46:41,600
names like capillary malformations or the old one “nevus flammeus.” These are another

623
00:46:41,600 –> 00:46:48,240
kind of birthmark that kids have. We see them not nearly as frequently as hemangiomas. Hemangiomas

624
00:46:48,240 –> 00:46:54,600
can pop up in about 5% of the population; port wine birthmarks pop up in only maybe

625
00:46:54,600 –> 00:46:59,960
one to two percent of the population at most, so it’s rarer but we do see these things

626
00:46:59,960 –> 00:47:05,960
and I’ll ask you how, what’s your approach to differentiating port wine birthmarks – by

627
00:47:05,960 –> 00:47:10,120
the way, they used to be called “port wine stains” if you’re confused listening. We’re trying

628
00:47:10,120 –> 00:47:14,880
to get away from using that word “stain” because it sounds negative and this is a kid’s

629
00:47:14,880 –> 00:47:20,480
birthmark on a child and half the goal here is to teach them to be comfortable in

630
00:47:20,480 –> 00:47:24,320
their own skin so to speak. So calling something a “stain” isn’t really the nicest thing so

631
00:47:24,320 –> 00:47:28,840
if you see that there’s an old phrasing of “port wine stain” yes we’re talking about the

632
00:47:28,840 –> 00:47:33,560
same thing, but what’s your approach, Dr. Piggott, to differentiating these things?

633
00:47:33,560 –> 00:47:37,600
So the most important thing is to get a history from the family as to whether or not it was

634
00:47:37,600 –> 00:47:43,280
present at birth. If it was not present at birth it is unlikely to be a port

635
00:47:43,280 –> 00:47:48,960
wine. When they’re young they can even look a little bit pink but later in life they look

636
00:47:48,960 –> 00:47:54,840
red. They don’t hurt the baby – the baby’s not bothered by them and in babies

637
00:47:54,840 –> 00:47:59,720
they’re flat. They don’t usually have a texture. They don’t have that strawberry look of a

638
00:47:59,720 –> 00:48:05,880
hemangioma and if you’re seeing a child later in life the parents will give you also a

639
00:48:05,880 –> 00:48:10,760
history that it kind of grows proportional to the baby’s growth. It doesn’t grow out of

640
00:48:10,760 –> 00:48:15,660
proportion. It doesn’t spread to other parts of the body. I think that’s a really critical

641
00:48:15,660 –> 00:48:20,600
point for the listeners…So, these these port wine birthmarks are present at birth, you’re

642
00:48:20,600 –> 00:48:24,960
saying, and they’re totally flat so the first clue that maybe you’re not talking

643
00:48:24,960 –> 00:48:31,120
about a port wine birthmark would be that if all of a sudden the area started to

644
00:48:31,120 –> 00:48:36,600
raise. That would really suggest at least to me maybe we’re talking about a hemangioma,

645
00:48:36,600 –> 00:48:41,800
but specifically a segmental hemangioma – one of these larger ones which not to put the

646
00:48:41,800 –> 00:48:46,440
fear of God into anybody but those are really an emergency in the sense that they can be

647
00:48:46,440 –> 00:48:50,920
related to those other syndromes that you mentioned. But port wine birthmarks themselves –

648
00:48:50,920 –> 00:48:57,040
they will tend to stay flat. You might see two weeks in they might get a little

649
00:48:57,040 –> 00:49:01,160
lighter. That’s sort of the physiological changes that happen in those new babies and

650
00:49:01,160 –> 00:49:05,800
then they go right back to being red. They also don’t as far as I’ve experienced they

651
00:49:05,800 –> 00:49:11,760
don’t fade. Yes and one of the things that you can confuse it with…

652
00:49:11,760 –> 00:49:17,880
there’s something called a “nevus simplex” which is they have all sorts of names for them: angel’s

653
00:49:17,880 –> 00:49:25,280
kiss,” “stork bite,” “salmon patch,” which are other red birthmarks that are flat at birth, present

654
00:49:25,280 –> 00:49:31,960
at birth, and they can be very commonly located on for example the eyelids. They can be in

655
00:49:31,960 –> 00:49:36,760
your glabella which is the lower part of your forehead almost in a v-shape and those are

656
00:49:36,760 –> 00:49:41,160
different in that they’re present at birth but very commonly fade over the first year

657
00:49:41,160 –> 00:49:46,160
of life and in most cases even go away completely where with the exception being the one at

658
00:49:46,160 –> 00:49:51,000
the back of the neck of course the stork bite which is different from a port

659
00:49:51,000 –> 00:49:57,200
wine birthmark, which will not really fade over time and quite conversely now it happens

660
00:49:57,200 –> 00:50:03,580
long long time not when the child is a baby or even a teenager but when you’re talking

661
00:50:03,580 –> 00:50:08,520
the patient becomes an adult we see port wine birthmarks not only not fade but we see them

662
00:50:08,520 –> 00:50:13,240
actually turn darker though they’ll assume a sort of a purplish hue which is where they

663
00:50:13,240 –> 00:50:19,560
get the term “port wine” to begin with and then they can also develop blebs -these little bumps

664
00:50:19,560 –> 00:50:24,760
and sort of thickening within them. Do you see that a lot? Yeah they are and again

665
00:50:24,760 –> 00:50:30,340
this is usually not until they’re teenagers or even later they thicken almost like

666
00:50:30,340 –> 00:50:35,160
a leathery textured feel but you will never see this in a child so when you’re trying

667
00:50:35,160 –> 00:50:39,800
to figure out if it’s this versus another type of birthmark you wouldn’t see this at

668
00:50:39,800 –> 00:50:43,640
a young age. That’s a great point. Yeah you would expect to see

669
00:50:43,640 –> 00:50:49,000
that maybe 40, 50, 60 years out. Now the good news is, you know, jumping to the punchline

670
00:50:49,000 –> 00:50:52,560
of what to do about these things – we’re not seeing a lot of those patients who are 50

671
00:50:52,560 –> 00:50:57,280
or 60 because we’ve got some amazing treatment options but before we get into the management

672
00:50:57,280 –> 00:51:03,920
approach what’s your current understanding of what’s driving these birthmarks, these

673
00:51:03,920 –> 00:51:10,360
port wine birthmarks? There’s actually a gene that we think might have a somatic mutation…

674
00:51:10,360 –> 00:51:17,740
it’s called the GNAQ gene which we think might be associated with some of the facial port

675
00:51:17,740 –> 00:51:25,200
wine stains especially those that develop into something called Sturge Weber but what

676
00:51:25,200 –> 00:51:32,000
What is your opinion? No I think that’s pretty pretty well studied now. Most of these

677
00:51:32,000 –> 00:51:36,360
birthmarks I think there’s a couple rare cases where the kind that are

678
00:51:36,360 –> 00:51:41,400
being passed from one family member down to another has been associated with RASA1

679
00:51:41,400 –> 00:51:47,260
mutations but by far when you do any of these sort of genetic studies

680
00:51:47,260 –> 00:51:52,480
on the tissue that constitutes these birthmarks you’re seeing the GNAQ mutation,

681
00:51:52,480 –> 00:51:58,080
like you mentioned. It’s very important to say though this is when you say somatic mutation

682
00:51:58,080 –> 00:52:02,880
this is not usually something that gets passed from mother or father to a baby. This is something

683
00:52:02,880 –> 00:52:08,320
that’s happening in the skin after the child was born and my limited understanding

684
00:52:08,320 –> 00:52:14,900
of the physiology or the pathophysiology of these is that the earlier that mutation

685
00:52:14,900 –> 00:52:18,640
happens it’s usually more associated with some of the bigger problems. What are some

686
00:52:18,640 –> 00:52:23,880
of the bigger problems that we see with port wine birthmarks? So the most important one

687
00:52:23,880 –> 00:52:34,440
to identify early is a syndrome called Sturge-Weber syndrome and the sort of classic case is that

688
00:52:34,440 –> 00:52:44,040
baby is born with a large red port wine birthmark on the forehead-upper eyelid area and

689
00:52:44,040 –> 00:52:48,360
the reason this is very important is because this is actually we call it a neurocutaneous

690
00:52:48,360 –> 00:52:55,760
syndrome. Not only does it have a facial port wine stain it can have leptomeningeal capillary

691
00:52:55,760 –> 00:53:01,160
or capillary venous malformations which is things in the brain and it can also have an

692
00:53:01,160 –> 00:53:06,920
increased risk of the baby having glaucoma. Other rare things that the syndrome can have

693
00:53:06,920 –> 00:53:13,640
the baby can have epilepsy, encephalopathy, and hemiparesis so when you see a baby with

694
00:53:13,640 –> 00:53:20,160
a large hemangioma especially on the forehead sort of upper eyelid eyebrow area we often

695
00:53:20,160 –> 00:53:27,040
do imaging such as an MRI to assess the brain and also we consult our pediatric ophthalmologist

696
00:53:27,040 –> 00:53:33,160
to assess for glaucoma for which early intervention is key to save the baby’s vision.

697
00:53:33,160 –> 00:53:37,340
Yeah you got to really be an advocate for the patient there and those wait times for

698
00:53:37,340 –> 00:53:42,400
pediatric ophthalmology can be really long as well but most pediatric ophthalmologists

699
00:53:42,400 –> 00:53:45,480
that I’ve ever worked with will take these kids very quickly and make sure that they’re

700
00:53:45,480 –> 00:53:46,480
okay.

701
00:53:46,480 –> 00:53:51,080
The good news is there’s treatment, which you are one of the top experts in.

702
00:53:51,080 –> 00:53:53,000
In treating these things…

703
00:53:53,000 –> 00:53:55,360
Yes, the laser..

704
00:53:55,360 –> 00:54:03,400
Well, thank you. I do enjoy it. It’s very rewarding to be able to have a piece of machinery – this

705
00:54:03,400 –> 00:54:10,000
pulse dial laser is what we’re talking about – here that will take a birthmark like this

706
00:54:10,000 –> 00:54:16,360
and not with infrequent amount of treatments – you need, you’re talking 10 15 maybe even 15

707
00:54:16,360 –> 00:54:22,240
to 20 treatments – but you can almost assuredly take these birthmarks from what

708
00:54:22,240 –> 00:54:28,120
they look like and reduce the amount of pink and red in them by about 75 to 85 percent

709
00:54:28,120 –> 00:54:34,080
with really not much difficulty and then going that extra little 10 to 15

710
00:54:34,080 –> 00:54:39,360
percent is really kind of where maybe the art is, but we’ve been able to do amazing

711
00:54:39,360 –> 00:54:43,440
things with this and that’s – actually it’s funny that you brought that up because I was

712
00:54:43,440 –> 00:54:47,640
able to train with one of your colleagues Vic Ross who’s out there at Scripps and

713
00:54:47,640 –> 00:54:54,100
he was very generous in teaching me what to do and how to do it so, yeah, we’re pretty thrilled

714
00:54:54,100 –> 00:54:57,720
to be able to have that technology and it hasn’t changed much in 20 years. I mean

715
00:54:57,720 –> 00:55:00,540
it’s really been the go-to machine.

716
00:55:00,540 –> 00:55:03,040
What does a laser do, Dr. Krakowski, to the blood vessel?

717
00:55:03,040 –> 00:55:08,400
Yeah, so it targets the blood – specifically the hemoglobin within the blood

718
00:55:08,400 –> 00:55:17,280
vessels – and the laser for a quick description is basically superheating very quickly

719
00:55:17,280 –> 00:55:23,040
and very focusedly the chromophore – this hemoglobin inside the blood vessel – which

720
00:55:23,040 –> 00:55:28,160
acts then to fry the blood vessel. It’s cauterizing – if you’re familiar with that term – it’s cauterizing

721
00:55:28,160 –> 00:55:33,920
the blood vessels from inside the blood vessels, which knocks them out and and keep the blood

722
00:55:33,920 –> 00:55:37,200
from flowing within them.

723
00:55:37,200 –> 00:55:40,320
The problem is those blood vessels tend to want to grow back and you have to keep going

724
00:55:40,320 –> 00:55:44,720
back in and knock them down, but but we’ve gotten very good at doing that and now there’s

725
00:55:44,720 –> 00:55:51,280
even some modalities where we’re treating much like PDT photodynamic

726
00:55:51,280 –> 00:55:58,040
therapy – we’re looking at taking a medicine infusing it into the patient’s bloodstream –

727
00:55:58,040 –> 00:56:03,480
this is preferentially picked up by a port wine birthmark because of the extravascular

728
00:56:03,480 –> 00:56:08,360
pressure that’s there and then taking a light source from outside and shining it onto the

729
00:56:08,360 –> 00:56:13,720
birthmark from the outside and you’re basically cooking this birthmark from within so

730
00:56:13,720 –> 00:56:18,920
it’s kind of a neat time to be involved with port wine birthmarks and being a part

731
00:56:18,920 –> 00:56:25,280
of that team but at the end of the day you have to really first make the diagnosis of

732
00:56:25,280 –> 00:56:32,000
what you’re dealing with. And I think in today’s show what we saw was not all red is either

733
00:56:32,000 –> 00:56:36,520
a port wine or a hemangioma. There are a lot of other things that these could be. I

734
00:56:36,520 –> 00:56:41,040
think that really speaks to the value of having a good medical team caring for your

735
00:56:41,040 –> 00:56:46,760
child – having great access to a team that has the expertise to be able to differentiate

736
00:56:46,760 –> 00:56:51,040
between these different conditions and thankfully there’s people out there like Dr. Caroline

737
00:56:51,040 –> 00:56:56,920
Piggott at Scripps and my pediatric dermatology colleagues who’ve devoted their entire lives

738
00:56:56,920 –> 00:57:03,360
to doing just that. So with that, Dr. Piggott, thank you! I can’t believe it’s our first

739
00:57:03,360 –> 00:57:07,640
show together and we’ve been able to finally get this to work but it was really fun.

740
00:57:07,640 –> 00:57:09,600
Thank you so much for having me..

741
00:57:09,600 –> 00:57:16,320
All right, we will see you soon.

742
00:57:16,320 –> 00:57:20,760
Thanks for tuning in to this episode of the Don’t Be Rash Pediatric Dermatology podcast.

743
00:57:20,760 –> 00:57:22,760
I’m your host Dr. Andrew Krakowski.

744
00:57:22,760 –> 00:57:26,880
Don’t forget to subscribe to our show on your favorite podcast platform and check out

745
00:57:26,880 –> 00:57:29,920
DontBeRash.org for more information.

746
00:57:29,920 –> 00:57:34,640
A special thank you to our nonprofit sponsor, the St. Luke’s University Health Network, for

747
00:57:34,640 –> 00:57:36,440
making this episode possible.

748
00:57:36,440 –> 00:57:57,480
Until next time remember: Keep calm and don’t be rash!