Welcome to the Don't Be Rash Pediatric Dermatology Podcast, the owner's manual for your kid's
skin.
I'm your host, Dr. K, board certified pediatric dermatologist and father of two boys.
I'm here to chat with you to promote dermatological education and improve skin health in our children
everywhere.
Let's get started.
Today's show is going to be a twofer.
We're going to try to cover infantile hemangiomas and port-wine birthmarks.
Joining me today as co-host and my very special guest is Dr. Caroline Piggott, renowned pediatric
dermatologist, a mother herself, accomplished figure skater, and one of the coolest people
I've ever had the pleasure of working with and really truly one of my best friends.
Dr. Piggott did her training in general dermatology with me out at the University of California,
San Diego.
We managed to stay together for our clinical fellowship in pediatric dermatology at Rady
Children's Hospital, San Diego.
Dr. Piggott's clearly smarter than me though, because she stayed there and now lives in
La Jolla and works at Scripps, one of the most beautiful places on earth.
Welcome, Dr. Piggott.
Thanks for joining me from across the country.
Good morning.
Thanks so much for having me, Dr. Krakowski.
It's good to see you.
It's been a long time.
We've been wanting to do this for a while now, so I'm so excited.
We finally get to put it to the test.
No, I'm glad to do it.
So Dr. Piggott, let's jump right in.
How do you approach a red birthmark in a child and, ultimately, how do you make that very
specific diagnosis of infantile hemangioma?
The babies present to me usually a couple of weeks of age with their parents who are
usually somewhat-to-very concerned.
The first thing I do is get a little bit of history.
So I ask them, was it there at birth or when did you first notice it?
Because one of the key things about hemangiomas is they present at some point, usually in
the first month of life.
Some of them are actually present at birth, but some of them are completely absent at
birth and present maybe one, two, three, four weeks of life.
And they often start out flat, like a little red mark.
Many parents will say to me they thought it was a bruise or they thought they had pinched
the child or something like that, and then they'll give me a history that it gradually
starts to get a little bit thicker and maybe darker.
Do you see them in any particular anatomic location or they can be all over?
Oh, they can be anywhere, head to toe really.
Sometimes parents won't even notice because it's in the private parts and they don't
really look very carefully or in some babies who are in the hospital, for example, if they're
born prematurely, they might be in the NICU.
Some parents don't even notice them because they're covered by leads or diapers or things
like that at first.
So they can really be anywhere.
Yep.
Up on the scalp.
If you're born with a bushy head of hair, you might not notice it.
Exactly.
Or parents will say, we thought it was just from the electrode on our child or something
like that.
We've been fooled a couple of times with, like you said, ones that are sort of between
the, for lack of a better word, the butt crack.
You don't know what's there.
Either the scrotum and a male child, you might, you could find them.
They can be anywhere really.
So do you see them always by themselves?
Is it an isolated thing or can you see these present in different ways?
So the most common form is when they're solitary and there's just one.
And that's what I see most commonly.
But there are actually cases where you can have multiple hemangiomas.
It's very important to count how many there are because there's a rare condition where
not only do you have hemangiomas on the skin, but also hemangiomas inside the body.
We call it systemic hemangiomatosis.
What I always do is, you know, the parents might not even know that there's other ones.
So what I always do is completely undress the baby and examine them head to toe.
And I actually count them.
The risk of having ones inside your body.
There's no clear consensus, but I would say on average if there's five or more on the
body, the risk of having one inside the body is a little bit higher.
And sometimes if there's five or more, or sometimes even four, because that's close
enough, we do actually imaging.
The most common type of imaging being an ultrasound of the abdomen because the most common area,
if you're going to have one inside the body, is actually the liver.
So an ultrasound is easy to do, no radiation, not harmful for the baby, no sedation needed,
and you can actually do an ultrasound to make sure there's not one in the liver.
And they can see them pretty easily on ultrasound.
Very easily.
I mean, when I get the reports back, it's either there or it's not.
It's very helpful.
Exactly.
And the reason that's important is because if, let's say, there was one in the liver,
if it's small, it doesn't really matter, but let's say there is one in the liver that's
large, it could be pushing on the rest of the liver and impact its correct function.
It could affect circulation.
And there's even some, I believe, data about affecting thyroid.
Yeah, it can definitely impact thyroid function, especially when you have diffuse hemangiomatosis
on the liver.
But...
Again, this is all very rare stuff.
Most of the time, even with multiple, there's nothing inside the body.
Yeah.
And most of the time, since you brought up the most usual presentation, most of the time
these are isolated.
I think one of the hardest parts for pediatricians and family medicine practitioners out there
and parents is trying to figure out to differentiate between a flat hemangioma, what we might call
like a superficial one, versus something like a port wine birthmark, which we're going to
talk about in the second half of this show.
But hemangiomas don't have to be flat when you first see them either.
Want to talk a little bit about how they might show up from the way they involve the skin?
So there's certainly ones that are flat.
And there are some that are actually raised above the skin.
And they can be quite significantly raised.
And those will look very red.
The reason why we call them "strawberry hemangiomas" is they often look like a strawberry.
There are also ones that can present under the skin, which makes our job a little bit
more challenging because when they're under the skin, they sometimes look blue.
Just like when we look at our veins, our veins are full of red blood, but under the skin
they look blue.
So that can sometimes make the diagnosis a little bit more challenging because there
are actually other things under the skin that can look blue that would be on the differential.
And I've had some hemangiomas where there's no color at all.
You have to get imaging and you find out, oh, it's a really deep hemangioma.
You just can't see the surface of the thing to even know there's blood in there.
So you can be fooled.
Now you tell me how you approach your patients, but my sort of go-to spiel is, listen, just
like you said, these things usually will follow a stereotypical course if we're lucky.
They're going to maybe not be there at birth.
They could be there at birth.
That's one of the things that helps us differentiate between conditions like congenital hemangiomas,
which are a little different than infantile hemangiomas, both in what they do, but also
maybe how they even got there in the first place, which we can talk about if there's
time.
But most of the time there's really nothing there at birth.
And then within, like I usually say, about a week or two, you'll see a cherry red spot
that then grows pretty quickly.
And I'll tell them to expect a lot of growth in the first three months, even up to like
five months.
I've seen some growth go.
And then these infantile hemangiomas will sort of transition to what is called the plateau
phase, where they're not doing anything.
They're just hanging out.
They're not getting bigger.
They're not getting smaller.
They're not causing any trouble.
And then fingers crossed around, I don't know, I tell people usually around 10, 11, 12 months
of age, fingers crossed, nature starts taking over.
And without doing anything, most of these birthmarks, these infantile hemangiomas, start
to want to go away on their own.
And if you're lucky, I usually quote that about half of them are gone by the time you're
five years of age.
Is that about your spiel as well?
Yeah.
I say maybe 20% are gone at age two, 30% age three, et cetera.
50% are gone at age five.
Sometimes after that, if at age five it's still there, there is a chance it could always
be there.
Certainly involution can continue up to even seven, eight, nine years of age.
But some of them actually don't resolve completely.
But the vast majority will get flatter, lighter.
Some completely disappear where you see literally no trace.
But the other thing that can sometimes happen is when they go through their growth phase,
they stretch out the tissue a little bit.
So even though they do get lighter and smaller over time, sometimes the hemangioma may be
gone.
But later in life, you see almost like a little pooch of the skin.
Yeah.
I caution my patients with hemangiomas that there's going to be, think of it like a "scaffolding,"
what someone's doing, putting a new roof on your home.
And to get that roof there in the first place, you need to build the scaffolding on the side
of the house that allows those roofers to get up top there and build their roof.
In this case, the roof are the blood vessels of the hemangioma.
And that scaffolding is that connective tissue that then hangs around and leaves, if everything
else goes away, it could leave behind this sort of residual, I think, or fibro fatty residual,
I've heard it called.
And that's a little harder to treat.
But it still can be treated.
And that's where the decision comes as to whether or not you want to treat based on
the location of the lesion, et cetera.
Because we do have treatment, of course.
And that's where I kind of begin to speak with the parents on, do we want to treat or
not?
Yeah.
So before we get into how we would actively manage these things, optimistically, what
do you tell patients and their families to look out for signs that these things might
be going away?
That they might be getting ready to get ready?
It's almost like a grape turning into a raisin.
Sometimes they start to sort of dry up, shrivel up, get a little flatter, lighter.
You see almost like a whitish discoloration.
And so that's, especially in involution phase, that's when I tell parents to watch out for
something called "ulceration," which is sometimes they dry up, shrink down so much that they
can even open up.
Yeah, and also in the rapidly growth phase, if you've got one that's really growing quickly,
you might see, classically they say there's like a white streak that you might see as
an impending sign of doom.
But yeah, I'll look for mottling where the color, that red color, a violaceous color
will turn, darker purple and start to break up a little bit.
And it gets softer too.
You can palpably feel it changing under your fingers, usually, that it's getting softer.
And man, even within a couple of days, sometimes you'll see a difference in terms of what this
thing looks like.
It's pretty magical.
Who's at risk for getting infantile hemangiomas?
So any baby could have them, but there is some data in the literature that shows that
it's a little bit more likely in a female baby.
Babies born prematurely.
And the reason for that, we don't completely understand, but there's some, there's a hypothesis
that might have something to do with a lack of oxygen compared to full term babies, but
I would say female and premature babies most common.
Do they still say twins are more at risk?
I think so, right?
Maybe, yeah, multiple gestation.
And then the question is also, could that be due to prematurity too?
Right, right, of course.
Low birth weight, I think is, that's probably one of the bigger things that I think of.
But we saw tons of them.
I mean, that's for sure.
We - you and I - trained and practiced in a time where the only treatments really were giving
it time and letting nature do its thing or putting kids on systemic steroids or even
worse, something like an anti-cancer medicine in the real bad cases.
And that's, we were, I think, I don't use the word lightly, but we were sort of blessed
to witness what, at least I tell people was the first miracle I've ever seen in medicine,
you know, the birth of oral propranolol.
Oh, yes, that was right when we were in residency that they discovered it.
And I remember before then we'd have babies on prednisone for months, which we know is,
you know, not an ideal situation.
I remember even ones where they were close to the eye, they used to have to inject steroids
into them.
So it was such a miracle.
Putting a needle into a kid's eye and hoping that the medicine you're injecting
is going to save their vision was a different experience.
And we were pretty fortunate.
We trained at a place where, you know, one of your colleagues there at Scripps, Sheila
Friedlander, MD - when she worked there - she was absolutely on the cutting edge of what was
going on in hemangiomas in general.
And then I think literally you and I were there when we were doing some of the biggest
research and clinical trials around the medicine that eventually came out to be the "gold standard"
for treatment, the stuff, oral propranolol.
And I think it's so amazing how they figured it out initially, too.
They were treating babies in a hospital.
Was it in Spain or something?
France, Bordeaux, France.
Who needed propranolol for, I think, cardiac indications.
And some of them happened to have hemangiomas and they started to notice that they were
getting better.
And that's how it all started.
To me, that lady who, I mean, granted, she got her respectful dues by getting a publication
out of New England Journal of Medicine.
And anybody who knows the story, you know, thinks she walks on water.
But just if you pull up at 50,000 feet and just understand what it took
for her to figure this out.
I mean, like you said, she had a kid on, from what I understand the story to be, was she
got called for a consult in the neonatal intensive care unit at the hospital that she was at
for a couple of kids with hemangiomas.
And just in parallel, they had been started on an oral beta-blocker, or probably actually
was probably systemic, I would think, because it was the NICU.
And she was able to then do two things, which I give her way more credit than what I would
have been able to do.
A, she pieced this all together.
And B, she did it because she actually went back very quickly to check on how these kids
were doing after she started them on oral steroids, and realized that,
geez, I think this is too soon.
The effect that we're seeing is too soon to have been the consequence of these steroids,
which we know took months and months and months to work.
And somehow she was able to put that together in her brain.
And to me, that is like, I don't know, at least within the world of dermatology, that's
Nobel Prize worthy, because she made that connection and then had the guts to look at
it more formally, write it up, submit it to the New England Journal of Medicine as a brief
report.
And now it's the gold standard.
And it's totally changed the way we do things, thankfully.
And so before we dive into management, because that's obviously one of the positive sides
of this discussion, you mentioned a little bit about how hypoxia may play a role.
Do you remember when you were training what the old thoughts were that this was caused
by?
I don't want to plant that in your head, but I kind of have a weird story in my mind of
being told what this was and going, oh, that's interesting, and then just buying it as totally
ex-cathedra, but then figuring out that down the line that that didn't make any sense.
Does that trigger anything?
No, no.
So I remember being told that this was probably a chunk of mom's placenta that got broken
off and was now being passed through the kid and was quote-unquote "growing."
Oh, and that's what the hemangioma is?
Oh, yeah.
I know.
I never heard that.
And I was, wow, my goodness, that sounds horrifying.
And then you're like, yeah, that's probably not really it.
But to your point, a lot of research has been done and this concept that hypoxia, that's
the technical term for it, but just lower oxygen levels in the tissue.
Not that the baby is suffering from any sort of low oxygen state, but the idea that the
skin itself took a little miniature "hit" in terms of how much oxygen was going through
it.
And then could it be compensating by then sort of bursting out with blood vessels that
try to get more blood to that area because that area particularly was lower in oxygen.
That to me not only makes sense, but it's backed up by a little bit of the science.
So we know these infantile hemangiomas, they're Glut-1 positive.
Can you speak at all about how you use Glut-1 as a marker for these things?
Yeah.
Glut-1 is "glucose transporter 1."
And one of the things we actually do in the clinic, for example, let's say we don't know
if it really is a hemangioma or it's like one of those subcutaneous ones and we're not
sure if it is one and that determines how we treat it.
If you actually take a biopsy of a hemangioma, you can actually stain it for that and that
can help you determine if it's a hemangioma versus some other sort of venous malformation
or whatever.
And if the biopsy is Glut-1 positive, you then know it is a hemangioma.
It's super specific to these particular things, right?
And it can be helpful too because there's some rare variants of hemangioma, not really
a variant but an alternative type of vascular lesion.
There's one called a congenital-type hemangioma and there's actually two different ones,
a non-involuting congenital hemangioma and a rapidly involuting congenital hemangioma that
look very similar but have different courses and one of the things we do to differentiate
a hemangioma of infancy from them is actually do the staining on a biopsy.
And, God forbid, there are a couple scarier "blue things" that can start to grow in kids
that we know about, tufted angiomas and let's see if I can say this one correctly.
The Kaposiform hemangioendothelioma.
Hachoo!
It's a mouthful.
But those two are famous because they can cause a devastating problem called Kasabach-
Merritt Syndrome where platelets get kind of stuck inside the lesion.
Totally something that we don't see in infantile hemangiomas but sometimes you don't know
and biopsying can make the difference for you.
But that said, when's the last time you biopsied a hemangioma?
Not often because hemangiomas are made of blood vessels and when you're in your outpatient
clinic without an OR you have the risk of bleeding.
So not my favorite thing to do.
Yeah I think we did it a couple times maybe during training but at least in the last five
years I have not stuck anything into a hemangioma.
Rather than I guess stuck on might be a way to phrase it, I think we're in a time now
where some of the management options that we have do afford us a week, a two weeks period
where we can actually say hey let's try these medicines that really should only work on
infantile hemangiomas.
It would be very helpful I think to have almost automatically when you're asking that ultrasound
a hemangioma would be great if the radiologist automatically did the flow.
Sometimes you have to go back and ask for that to be done.
I don't know about your institution but.
We do have to ask.
One of the things I wish we had been trained to do it ourselves even.
Oh that would be great right?
Right at the bedside would save a lot of people a lot of worries but ultimately we get the
answer and it is very reassuring when you see that it's a "fast flow" lesion
not "pulsatile" like you said in arteriovenous malformation in AVM. Not a slow flow lesion
like a venous malformation but the blood going through these tiny little capillaries that
make up this what really is a tumor of blood vessels, right?
That's a big thing that and a scary thing to hear for parents but when you're kind of
looking at red birthmarks on a kid the way at least I kind of characterize them and make
the first split...is this a tumor of blood vessels or is this a malformation of some vasculature
and hemangiomas fall into what is technically a bucket of tumors in the sense that it's
not a cancer it's not going to spread and take over the person's body but it's a tumor
it's not supposed to be there and it's living growing tissue that has sort of connected
itself in a place that's not supposed to be.
Speaking of places where it's not supposed to be what anatomic locations get you nervous?
Well the two main ones would be face with that having both medical and cosmetic implications
and then the second one would be the genital area and there's a couple of reasons let's
start with the face so I always tell my patients there's two issues medical and cosmetic so
medically on the face especially in places for example can you imagine on the eyelid
where a hemangioma might grow and push on the eye or impede the baby's ability to open
their eye that can affect vision.
On the nose...imagine, you know, it being near the airway. It can affect a baby's ability
to breathe. And then certainly on the mouth...the baby's ability to feed and
then another consideration would be the size of the hemangioma because there's this rare
condition we call it PHACE syndrome where you have a large hemangioma commonly kind
of in this distribution of the "beard" area there's a condition called PHACES syndrome.
P-H-A-C-E stands for the P is for posterior fossa malformations
The H is for hemangioma. The A is for arterial anomalies. The C is for cardiac defects or
also actually aortic coarctation, and the E is for eye anomalies and the most common location
for this syndrome is to have a hemangioma on the face so I don't know what your experience
with those has been.
We've had a couple over the last five years that we've had St. Luke's Dermatology up
and running, and they they've ranged from being caught early on and being effectively
managed now with the oral propranol that we have to having some kids that have had consequences
where you know it would have been impossible to prevent but that they've had some of those
other findings that you mentioned. More specifically, the the heart stuff is seemingly
what we tend to find, not - thank God! - not a lot of brain issues or but the but they'll
have some heart associated defect and you kind of just lump that in together and say
this would be consistent with PHACE syndrome even if it's totally unrelated
because we don't have a way to test both directly and say oh yeah this is the this is because
of the same thing.
And not only for the facial hemangioma is the medical side, which is clearly the most
important there's the cosmetic side too. I mean you have this large tumor growing on
your beautiful child's face.
It can distort the tissue we know that they involute in the future but you might be you
know left with stretched out tissue right in the middle of your face so and which is
very concerning.
Yeah so I kind of approach it just exactly like you have...is this hemangioma
going to pose a functional risk and will it pose a long term cosmetic risk. And I think
you hit it on the head... Eyes. So we have a wonderful pediatric ophthalmologist that we work with
that gets these kids in very quickly, makes sure that the optic nerve is intact that there's
no findings of PHACE. If there is one that's growing close to the eyelid margin you could
imagine if you're catching that early enough that you're going to maybe see some rapid
growth over the next few weeks. This thing could grow literally up and into the field
of vision so - it's really interesting! - the ophthalmologist will have - and I only learned this by training
in pediatric dermatology but for the people out there who never would have thought this
through you actually in those cases - you will purposefully have the child block the unaffected
eye, the eye where the hemangioma is NOT. And that forces the kid to use the vision of the
eye that is being somewhat interrupted by the hemangioma and that keeps those optic
nerve pathways intact and alive, because in pediatrics there is this true phenomenon of
"Use it or lose it!" Right? So you want to keep both of those eyes working
equally and it's kind of cool when you get those kids in and you can make a
real difference for them long term. Nose, for sure. we've involved pediatric Ears, Nose and
Throat (ENT) in a bunch of cases and probably even more so for the "beard" area. Yeah, how about
the "beard" area? You want to chat a little bit about that? It can actually push on your airway
or esophagus so and there's some cases where the hemangioma itself might clinically appear
from the outside is completely flat but actually has a deeper component. I remember a case that
in residency where a what we thought was previously just a flat hemangioma the child
presented to the ER with stridor and after imaging and scoping by ENT it turns out it actually
was pushing much below and of course we could fix that with propranolol but it was
quite scary. Yes, what's stridor for the audience? It's a funny sound that babies will
will make almost like a...Can you replicate it? Yeah, yeah almost like...GASP. Maybe, is that
pretty good stridor? Yeah I don't know. Yeah it's just almost like gasping and and it's
frightening because the the hemangioma can grow really quickly and actually require the
baby to be intubated if not treated quickly. Yeah so for anyone listening out there
if you happen to have an infantile hemangioma on your child in the sort of the
"beard" area where you might be able to grow a beard that's a clue for
a kid - a baby. You can't put them on a treadmill and do a "stress test" so for the
kid that stress test is usually eating. They're chugging away on the bottle or the breast
and they're really using all of their energy and so if you've got one
of those birthmarks in that area and you're hearing your child make a sound that suggests
he or she's having trouble breathing that can be a real big sign that you need
to get in there and see somebody and not be told that you'll get into the dermatologist
nine months from now. You got to just show up at the door and make sure someone sees
you pretty quickly. One other area that I forgot to mention, too, is the ear. What could seem
like a superficial hemangioma can actually have a deeper component and affect the development
of your ear or hearing of your child. So cosmetically I've actually had two patients now where if
the top of the ear for lack of a better anatomical description is involved that the
ear itself sometimes there'll be a segmental hemangioma that's part of that ear but
then also kind of spills onto the scalp and in those particular cases it really did deform
the top of the ear. In one child that sort of ulcerated and left the child with almost
like a bite out of the top of her ear, and the other child was left with sort of a bent
ear lobe as a result of that so that's a really important both functional and cosmetic area
for sure. How about in the diaper area? Where do you get into trouble with hemangiomas? So
same issue medical first most important depending on how big it is or where it's located it
can affect a baby's ability to urinate or stool. I've had a baby where
it completely impacted the perianal area and we had to do propranolol so the baby could
even have a bowel movement. You know it was blocking their ability to
poop. Yes okay yes and there's also a rare condition especially with a very large hemangioma
we call it a segmental hemangioma. There's sort of the the opposite of PHACE syndrome
in the area we call it LUMBAR syndrome: L U M B A R. Basically it's a lower body hemangioma
is the L. The U stands for urogenital anomalies and ulceration. The M stands for myelopathy.
The B stands for bony deformity excuse me. The A stands for anorectal malformations
of which there can be multiple and also arterial anomalies, and then the R stands for renal
anomalies.. And so I actually had one in clinic in the last year who presented with a large hemangioma
basically from the top of the labia all the way to the buttocks on both sides had to be
worked up for this. And what we do is some MRI imaging to check not only the urogenital
area also the lower spine and fortunately this baby turned out to be totally fine. It
was an isolated hemangioma but you have to look for these other anomalies. Yeah that
must have been horrifying. Thankfully that's pretty rare correct? And and so
once you rule that out the main issue in that area is then because you know you can imagine
having a large growth in your diaper what's the main issue that happens? You have you know
stool, urine in the diaper and you can get actually erosions, ulcerations which present
not only in a very painful way but also at a high risk for infection and bleeding. My
spiel for that is, you know, if you think about it urine's got ammonia in it and all the digestive
enzymes that start from your mouth all the way down to your anus winds up getting dumped
into the diaper. These diapers are right up against the skin. Even without a hemangioma
you can get "diaper dermatitis" a diaper rash where the real underlying cause is simply
just an irritation from those materials up against the skin. Now you're putting those
same caustic chemicals onto not normal skin but this infantile hemangioma skin - segmental
or otherwise. It can just be a normal little hemangioma and, man, I find that that
skin is particularly friable and will ulcerate a little bit more easily. So what happened
when we're talking about ulcerating...You want to explain what sort of that phenomenon is?
What do we mean when we say a hemangioma ulcerates? So for example in the growth phase as they
get larger sometimes the superficial portion of the lesion actually almost looks
like a small tear of the skin as it stretches out
and then it'll gradually open up and make a sore that can actually get wider and deeper
over time as the hemangioma grows and you can imagine having a sore rubbing against
the diaper. It's very painful one of the things I think parents should hear is that yes this
is for lack of a better description a big tumor full of blood but when when we get an
ulceration when the skin breaks down it's not going to show up as a bursting water balloon
of blood. That's what all the parents think is going to happen. Yeah sometimes they miss
it. They miss the fact that the skin that's overlying these things is getting eroded and
chewed up and is basically like you skin your leg. You can look for a surface change
that's really what we're talking about and, man, it hurts when you have
any break in your skin. A paper cut even feels not great to an adult. Imagine what this would
feel like in your diaper area for a little baby. On top of that like you said any of those
microbes especially in the diaper area from the poop are getting now into the skin when
it normally shouldn't. Anything can get in there and cause an infection which then can
really exacerbate all those problems so we we take it very very seriously and act very
aggressively when an ulcerated hemangioma presents. What's your sort of... what do you do
extra in terms of management for those kinds of things? You mean apart from using things
like propranol? Yeah yeah. Well, what else would you do for those? So I tell the
parents how important it is to keep the area well hydrated. We have various different brands
of petrolatum-based products that you can use in the area so I I counsel with every
diaper change you may want to put a layer on top as well as a barrier cream something
for example with zinc oxide to protect the hemangioma from rubbing against the diaper.
That's great. We've even had to prescribe topical lidocaine at times, and
we use metronidazole a lot for when it's in the diaper area just to try to control some
of the poop associated bacteria. I don't know if that's something you guys do but same thing.
But since we've been beaten around the bush here now what would your "go to" be
to treat any hemangioma where it's functionally at risk for causing problems or cosmetically
at risk for causing problems? What's your sort of management approach? So what I
do is I introduce the family to the concept of there being both an oral and a topical
beta-blocker available. The oral - the most common one we use is propranolol - and the topical being
timolol and what I present it as is an opportunity to shrink the hemangioma down in size and
it also improves the color but also helps lower the risk of development of these ulcerations
But the most important thing I talk about with families is that before starting anything
like this the decision has to be made as to whether you want to do the oral versus the
topical based on the risk and with those risks being the most important ones being the risk
of lowering your baby's blood pressure pulse and blood sugar and you and
the problem with that is these are babies and not adults an adult's blood pressure is
you know 120 over 80 a baby's blood pressure might be 80 over 50 at baseline and the problem
with lowering a blood pressure is then you don't get enough blood profusion to vital
organs like your brain so you have to be very careful with these medications. We don't just
give them to every baby. There there especially in smaller ones there is opportunity to start
with for example a topical version of this which is the timolol which has a lower risk
of those side effects although not zero and to a couple more side effects that are very
rare if a baby has asthma you could increase the baby's risk of wheezing if the baby. So
there's this rare thing we call night terrors that you can get with beta-blockers which
is basically in a nutshell the baby wakes up from sleep screams shouts falls back asleep
right away, which is very hard to differentiate from normal baby behavior at that age anyway.
But I always tell parents if it seems like it's happening more often it could be the
medicine and then I've also had a couple rare GI side effects there are reports of
constipation but I've also had actually some have the opposite effect. I don't know what
your experience is absolutely and and so reflux as well the baby's like spinning up a little
bit more and also vaso vasoactive changes so that like where their hands look cold. You
mean yeah yeah look like they went into a cold room and everything turned blue for a
couple seconds and then which is scary for a mother or father yeah it would look like
your kids suffocating you know it through his hands or feet but in really in real life
there's no known consequence when that happens it's just like a thing that's known to happen
with these medicines and I think you hit on a great point so these are blood - this propranolol
and so oral propranolol and topical timolol...topical by the way when we say topical what
we're talking about is you do not give this to your baby's mouth. You would be putting
it directly on the skin and in fact just as a little interesting side note from what I've
learned of topical timolol...It cannot be given - should never be given - orally because it's
actually much stronger in terms of its potency than oral propranolol and for that
reason I always tell my families who are using the topical you want to be very
certain who's giving this medicine - not a child that's helping take care of the his baby brother
or sister, not a mother-in-law who's visiting and forgets that this is not to be delivered
into the baby's mouth with the little dropper. This is going on the skin. That's what we're
talking about topical. It's actually an eye drop as its original function was for glaucoma.
Right yeah. Absolutely. It lowers the blood pressure in a condition called glaucoma and
that's the point so these are blood pressure lowering medicines being used in
a population of kids with infantile hemangiomas who don't really normally have high blood
pressure so all the side effects are directly related to taking this anti high blood pressure
medicine when you don't need those medicines. So like you said lowering your blood pressure
that's the probably the biggest risk you can cause someone to to to drop their blood pressure
dangerously low. You can die from that. You can drop their blood sugar levels dangerously
low. You can die from that.
You can get a seizure too.
Seizures yeah so it's not to be taken lightly. But at the end of the
day and I used to have it when we actually I don't know if you remember but when this
protocol first came out when we were fellows we had to call consults on the Cardiology
team - the Pediatric Cardiology team - every patient that we wanted to start this medicine on and
I had for a while I had it with me I think when I left Rrady Children's - I think it died
in the ethos - but I had a letter from one of the cardiologists there that said, "Please, please...
for the love of God stop calling me. The doses that you guys are maxing out at are not
even the doses that we usually start our kids on when they do have high blood pressure!" So
probably we were making a bigger deal of it but truthfully that was not something that
we were experts at back then. Now, thankfully, we've got what 12, 13 years of experience using
this medicine and I actually heard for the first time in our area a pediatrician was
managing this himself or herself without a dermatologist so it's sort of it's
starting to trickle down and I think people are less afraid and realize like with some
good prep time and preparation for the families you can avoid 98% of these
issues. Some are going to happen no matter what but the hypoglycemia...what do you
tell your patients to do to avoid hypoglycemia? You must always give
the medicine with a feed so what I do is I have the mom maybe give a half feed then give
the medicine then finish and the reason I don't do a full feed is sometimes they're
so full they don't even want to take the medicine. And then the other thing I caution is if a
baby is ill like let's say they have the flu - they're vomiting or they're not stooling normally,
or just not feeling good for whatever reason - I have the family skip the dose that
day. There's no harm to the hemangioma and taking a break for a day or two and it's not
worth the risk. Very similarly I always counsel the families like look - let's say little Mary
or a little Johnny is sitting there. You're getting ready to dose otherwise looks totally
healthy. You give the dose of propranolol and we almost exclusively use and it's one
of the only times we actually do almost exclusively use the brand name oral Hemangeol, which is
a very specifically studied form of generic propranolol. It's the same medicine but it
comes in a "twice a day" formulation rather than a three times a day. There's no alcohol,
which I think is a big plus for dosing babies, and it's flavored. It's supposed to be strawberry
vanilla. I never tried it myself but it's pink and my point here is when you give this to
the baby little Johnny, little Mary sitting there you give them the two mLs or whatever
their dose is supposed to be...They look at you. They smile and they puke it right back up
onto the floor - what's clearly six mLs, three times as much! What do you do? and I'll
just pause and I just say what would you guys do and usually someone in the room says do
nothing. That's the right answer! Don't give the dose again. Don't double up on it. You assume
little Johnny, little Mary got the entire dose into their brains and you're not going to
risk giving them a second dose right then and there. Just wait. If you miss one dose - to
your point, Dr. Piggott - who cares? It's not a matter of life and death. And
I also - one of the other things - especially in families with multiple caregivers I I remind
them how easy it is to forget that maybe dad gave it in the morning and mom doesn't realize
it and gives another dose, so I I encourage them to have like a little calendar on the
wall where it's checked off so you know that the dose was given or I usually or I'll say
you have just one person be in charge of giving this dose. That's brilliant! Yeah I like that
a lot. That has happened. Usually for these patients I'm giving them my personal cell
phones letting them know hey if you have any questions especially in the beginning but
two or three months in they're experts. But there are - and I did one Saturday get a call
from mom that dad had doubled the dose by not remembering that mom had done it so
it's kind of scary but at the end of the day baby's fine, healthy, eating, give him a couple extra
rounds of milk and don't give the next dose and you're fine. So the other thing to
remember is we're not starting this medicine "cold turkey," right? We're not going right up
to a dose that we would need to see clinical effect on these hemangiomas at least
we're not I don't know if you're still doing the ramp up but...Absolutely! You are? So good.
So the first week or so is a test dose that's not even supposed to do anything to the hemangiomas.
It's just supposed to be there to see how the kid's reacting to the medicine and then
once you establish that the heart rate and the blood pressure are still within healthy
limits then you actually give the second dose which you could expect to see some clinical
effect. If you don't there's even a third level which I sometimes don't even go up to
if I'm getting good effect at the second level I don't even go up to the third level. How
about you? Agreed and what we sometimes do if the baby is especially in let's say it's
a newborn like two weeks old rapidly growing you know PHACE syndrome type baby after we've
done the workup we there are cases where we even send them to the hospital to be
admitted for monitoring when they're started on the Hemangeol or the generic
propranol. Yeah and you know you and I were were just featured in that Practical Dermatology
roundtable that we got to do together, which is kind of cool but we talked a little bit
about how long these kids are on propranolol - when you start to take them off. What's
your approach there? Well it totally depends on on the case. What I try to do clinically
is when I see that the hemangioma is starting to involute on its own I might either leave
the baby at the same mLs and not adjust the dose for weight gain or sometimes I even go
quicker. There's actually an article published in the last year or two that suggested approximately
thirteen months or so is when a lot of people start to taper but I actually think I've done
it younger than that. Okay I tend to maybe even on the other side of things push a little
closer to a year year and a half especially depending on what anatomical area it's at
but I think for the purposes of this discussion - if anybody's out there and wondering if A...
their child has an infantile hemangioma and B are they at risk for any of these problems
the the real key point of this discussion is don't allow yourself or your family to be
told you'll get in to see the specialist in six months, nine months. Literally you know
if you have to you insist with the pediatrician, you insist with your family medicine doctor,
your primary care doctor whoever that may be...You say, "Listen I I need to get this child
in to make sure this is okay!" and then you reach out to the dermatologist or if you're
lucky enough to have a board certified pediatric dermatologist. There's not a lot of us out
there but they're enough that most of the time we are tuned in and our staffs tuned
in to know, hey, this could be a real emergency and we make every effort to get these kids
in and be seen quickly. So, with that I think for the last portion of the show we'll switch
gears just a little bit. We're still talking about red birthmarks but I want to
focus, Dr. Piggott, on port wine birthmarks. We know these things also go by a couple of
names like capillary malformations or the old one "nevus flammeus." These are another
kind of birthmark that kids have. We see them not nearly as frequently as hemangiomas. Hemangiomas
can pop up in about 5% of the population; port wine birthmarks pop up in only maybe
one to two percent of the population at most, so it's rarer but we do see these things
and I'll ask you how, what's your approach to differentiating port wine birthmarks - by
the way, they used to be called "port wine stains" if you're confused listening. We're trying
to get away from using that word "stain" because it sounds negative and this is a kid's
birthmark on a child and half the goal here is to teach them to be comfortable in
their own skin so to speak. So calling something a "stain" isn't really the nicest thing so
if you see that there's an old phrasing of "port wine stain" yes we're talking about the
same thing, but what's your approach, Dr. Piggott, to differentiating these things?
So the most important thing is to get a history from the family as to whether or not it was
present at birth. If it was not present at birth it is unlikely to be a port
wine. When they're young they can even look a little bit pink but later in life they look
red. They don't hurt the baby - the baby's not bothered by them and in babies
they're flat. They don't usually have a texture. They don't have that strawberry look of a
hemangioma and if you're seeing a child later in life the parents will give you also a
history that it kind of grows proportional to the baby's growth. It doesn't grow out of
proportion. It doesn't spread to other parts of the body. I think that's a really critical
point for the listeners...So, these these port wine birthmarks are present at birth, you're
saying, and they're totally flat so the first clue that maybe you're not talking
about a port wine birthmark would be that if all of a sudden the area started to
raise. That would really suggest at least to me maybe we're talking about a hemangioma,
but specifically a segmental hemangioma - one of these larger ones which not to put the
fear of God into anybody but those are really an emergency in the sense that they can be
related to those other syndromes that you mentioned. But port wine birthmarks themselves -
they will tend to stay flat. You might see two weeks in they might get a little
lighter. That's sort of the physiological changes that happen in those new babies and
then they go right back to being red. They also don't as far as I've experienced they
don't fade. Yes and one of the things that you can confuse it with...
there's something called a "nevus simplex" which is they have all sorts of names for them: angel's
kiss," "stork bite," "salmon patch," which are other red birthmarks that are flat at birth, present
at birth, and they can be very commonly located on for example the eyelids. They can be in
your glabella which is the lower part of your forehead almost in a v-shape and those are
different in that they're present at birth but very commonly fade over the first year
of life and in most cases even go away completely where with the exception being the one at
the back of the neck of course the stork bite which is different from a port
wine birthmark, which will not really fade over time and quite conversely now it happens
long long time not when the child is a baby or even a teenager but when you're talking
the patient becomes an adult we see port wine birthmarks not only not fade but we see them
actually turn darker though they'll assume a sort of a purplish hue which is where they
get the term "port wine" to begin with and then they can also develop blebs -these little bumps
and sort of thickening within them. Do you see that a lot? Yeah they are and again
this is usually not until they're teenagers or even later they thicken almost like
a leathery textured feel but you will never see this in a child so when you're trying
to figure out if it's this versus another type of birthmark you wouldn't see this at
a young age. That's a great point. Yeah you would expect to see
that maybe 40, 50, 60 years out. Now the good news is, you know, jumping to the punchline
of what to do about these things - we're not seeing a lot of those patients who are 50
or 60 because we've got some amazing treatment options but before we get into the management
approach what's your current understanding of what's driving these birthmarks, these
port wine birthmarks? There's actually a gene that we think might have a somatic mutation...
it's called the GNAQ gene which we think might be associated with some of the facial port
wine stains especially those that develop into something called Sturge Weber but what
What is your opinion? No I think that's pretty pretty well studied now. Most of these
birthmarks I think there's a couple rare cases where the kind that are
being passed from one family member down to another has been associated with RASA1
mutations but by far when you do any of these sort of genetic studies
on the tissue that constitutes these birthmarks you're seeing the GNAQ mutation,
like you mentioned. It's very important to say though this is when you say somatic mutation
this is not usually something that gets passed from mother or father to a baby. This is something
that's happening in the skin after the child was born and my limited understanding
of the physiology or the pathophysiology of these is that the earlier that mutation
happens it's usually more associated with some of the bigger problems. What are some
of the bigger problems that we see with port wine birthmarks? So the most important one
to identify early is a syndrome called Sturge-Weber syndrome and the sort of classic case is that
baby is born with a large red port wine birthmark on the forehead-upper eyelid area and
the reason this is very important is because this is actually we call it a neurocutaneous
syndrome. Not only does it have a facial port wine stain it can have leptomeningeal capillary
or capillary venous malformations which is things in the brain and it can also have an
increased risk of the baby having glaucoma. Other rare things that the syndrome can have
the baby can have epilepsy, encephalopathy, and hemiparesis so when you see a baby with
a large hemangioma especially on the forehead sort of upper eyelid eyebrow area we often
do imaging such as an MRI to assess the brain and also we consult our pediatric ophthalmologist
to assess for glaucoma for which early intervention is key to save the baby's vision.
Yeah you got to really be an advocate for the patient there and those wait times for
pediatric ophthalmology can be really long as well but most pediatric ophthalmologists
that I've ever worked with will take these kids very quickly and make sure that they're
okay.
The good news is there's treatment, which you are one of the top experts in.
In treating these things...
Yes, the laser..
Well, thank you. I do enjoy it. It's very rewarding to be able to have a piece of machinery - this
pulse dial laser is what we're talking about - here that will take a birthmark like this
and not with infrequent amount of treatments - you need, you're talking 10 15 maybe even 15
to 20 treatments - but you can almost assuredly take these birthmarks from what
they look like and reduce the amount of pink and red in them by about 75 to 85 percent
with really not much difficulty and then going that extra little 10 to 15
percent is really kind of where maybe the art is, but we've been able to do amazing
things with this and that's - actually it's funny that you brought that up because I was
able to train with one of your colleagues Vic Ross who's out there at Scripps and
he was very generous in teaching me what to do and how to do it so, yeah, we're pretty thrilled
to be able to have that technology and it hasn't changed much in 20 years. I mean
it's really been the go-to machine.
What does a laser do, Dr. Krakowski, to the blood vessel?
Yeah, so it targets the blood - specifically the hemoglobin within the blood
vessels - and the laser for a quick description is basically superheating very quickly
and very focusedly the chromophore - this hemoglobin inside the blood vessel - which
acts then to fry the blood vessel. It's cauterizing - if you're familiar with that term - it's cauterizing
the blood vessels from inside the blood vessels, which knocks them out and and keep the blood
from flowing within them.
The problem is those blood vessels tend to want to grow back and you have to keep going
back in and knock them down, but but we've gotten very good at doing that and now there's
even some modalities where we're treating much like PDT photodynamic
therapy - we're looking at taking a medicine infusing it into the patient's bloodstream -
this is preferentially picked up by a port wine birthmark because of the extravascular
pressure that's there and then taking a light source from outside and shining it onto the
birthmark from the outside and you're basically cooking this birthmark from within so
it's kind of a neat time to be involved with port wine birthmarks and being a part
of that team but at the end of the day you have to really first make the diagnosis of
what you're dealing with. And I think in today's show what we saw was not all red is either
a port wine or a hemangioma. There are a lot of other things that these could be. I
think that really speaks to the value of having a good medical team caring for your
child - having great access to a team that has the expertise to be able to differentiate
between these different conditions and thankfully there's people out there like Dr. Caroline
Piggott at Scripps and my pediatric dermatology colleagues who've devoted their entire lives
to doing just that. So with that, Dr. Piggott, thank you! I can't believe it's our first
show together and we've been able to finally get this to work but it was really fun.
Thank you so much for having me..
All right, we will see you soon.
Thanks for tuning in to this episode of the Don't Be Rash Pediatric Dermatology podcast.
I'm your host Dr. Andrew Krakowski.
Don't forget to subscribe to our show on your favorite podcast platform and check out
DontBeRash.org for more information.
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Until next time remember: Keep calm and don't be rash!