Abnormal Baby Heads – PediaCast 368

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Show Notes

Description

• Dr Gregory Pearson stops by the PediaCast Studio to talk about abnormalities of the infant head. We consider microcephaly and macrocephaly, along with the diagnosis and management of positional plagiocephaly and craniosynostosis. Big complex names, yes; but also fairly common problems. We hope you can join us!

Topics

• Abnormal Baby Heads
• Microcephaly
• Macrocephaly
• Benign Familial Macrocephaly
• Positional Plagiocephaly
• Craniosynostosis

Guest

• Dr Gregory Pearson
• Plastic & Reconstructive Surgeon
• Director, Center for Complex Craniofacial Disorders
• Nationwide Children’s Hospital

Links

• Center for Complex Craniofacial Disorders
• Positional Plagiocephaly at NCH
• Craniosynostosis Program at NCH
• PediaCast 323 – Baby Problems

Transcription

Announcer 1: This is PediaCast.

[Music]

Announcer 2: Welcome to PediaCast, a pediatric podcast for parents. And now, direct from the campus of Nationwide Children's, here is your host, Dr. Mike.

Dr. Mike Patrick: Hello everyone, and welcome once again to PediaCast. It's a pediatric podcast for moms and dads. This is Dr. Mike, coming to you from the campus of Nationwide Children's Hospital. We're in Columbus, Ohio.

It is Episode 368 for February 8th, 2017. We're calling this one "Abnormal Baby Heads". I want to welcome everyone to the program.

The title of our show this week pretty much sums up the topic. Abnormalities of the baby head. I know it sounds a little funny but it's as simple as I could put it.

Normally, I try to call out the conditions that we're going to be covering in the title of the program. But the conditions associated with an abnormal baby head, they tend to be big, long, complicated names. Things like microcephaly and macrocephaly which, okay, those aren't too bad. One means a small head, the other means a large head. But also plagiocephaly, which is an extremely common problem but I bet you never heard that name before. And it's not really a more common name that everyone uses for, it's just plagiocephaly.

Then there's craniosynostosis, which is not quite as common as plagiocephaly but still an extremely important thing to consider because failure to recognize and address it, can result on some pretty big problems down the road.

And so, I didn't think as a title that microcephaly, macrocephaly, plagiocephaly and craniosynostosis would've worked very well. You probably would have gone running in the opposite direction. Maybe not even listened because you had no idea what I was talking about. So, I went with abnormal baby heads, descriptive, yes and hopefully raises your curiosity to just the right degree. And I think it worked because you're still listening.

0:02:24

By the way, if the word craniosynostosis, if it sounds a little bit familiar, back in Episode 323, we had a listener question about craniosynostosis. And it was part of a collection of listener questions all pertaining to baby problems.

So, in that one, we also covered infant digestion, retractile testicle or testes that kind of go up inside and it's a little difficult to get them to come down into the sack. If you have a little baby boy at home, you may have dealt with that in the past. We also talked about fluoride supplementation and sudden infant death syndrome and baby sleep.

So, if any of those topics sound interesting, be sure to check out PediaCast Episode 323. And to make it as easy as possible to find, I'll include a link to it in the Show Notes for this episode, 368, over at PediaCast.org.

I covered craniosynostosis in that episode. But we talked about it very briefly and in the context of our listener's question. So, today, we're going to talk about the problem in more detail with an expert at diagnosing and treating the disorder.

Dr. Gregory Pearson is my guest today. He's a plastic and reconstructive surgeon at Nationwide Children's. Also director of our Center for Complex Craniofacial Disorders. He'll enlighten us on the ins and outs of craniosynostosis, which in a nutshell occurs when the infant's skull starts fusing too soon. The soft spot goes away too early and the skull can't grow properly.

Of course, there's lots more to it than that and we'll dive deeper and explore the issue in a few moments.

The other big word I mentioned 'plagiocephaly', that occurs when your baby develops a flat spot on the skull, often from lying in the same position for sleep. It occurs very commonly on the back or side of the head, especially since we've been recommending that babies sleep on their backs to prevent Sudden Infant Death Syndrome. So their head usually rests in the same position for hours at a time, night after night, and during naps. So a flat spot is prone to develop.

0:04:38

So the questions becomes are these flat spots or plagiocephaly, is it a problem? If so, what do you do about it?

You may have seen babies wearing helmets to fix the flat spot. Are those absolutely necessary? If so, why? If not, how do you decide to use one or not? Sometimes, you'll hear folks say, "No, they're a waste of money and they don't really help at all." So, we'll put Dr. Pearson on the spot and ask for his opinion on baby helmets to fix flat spots.

We'll also talk about microcephaly, which again is too small of the head, and macrocephaly, too big of a head. You guessed it. What causes those conditions? How are they diagnosed?

So lot's coming your way today as we consider the baby head and some of its associated abnormalities. Only at pediatric podcast, right?

But hey, it's a great time for explaining things. Even though it might be on your family's radar at this particular moment, but there will be folks coming along who search the Google machine for abnormal baby head or plagiocephaly or craniosynostosis. So, we're here for those parents, too.

And if you happened to be one of those moms and dads who found us today because of our topic. Welcome. We have lots of more shows for you on an incredible wealth of topics. Ten years of episodes as it turns out, so be sure to explore the archive of past of episodes over at PediaCast.org.

Or pick a topic and Google that word along with PediaCast and let Google do the work of finding one of our programs for you.

0:06:12 If we don't a topic that you'd like to hear more about, drop me a line with a topic suggestion or pediatric or parenting questions. I'd love to hear from you. It's easy to get in touch, just head over to PediaCast.org and click on the Contact link.

Also, I want to remind you the information presented in every episode of our program is for general educational purposes only. We do not diagnose medical conditions or formulate treatment plans for specific individuals. If you have a concern about your child's health, be sure to call your doctor and arrange a face-to-face interview and hands-on physical examination.

Also, your use of this audio program is subject to the PediaCast Terms of Use Agreement which you can find at PediaCast.org.

All right, let's take a quick break. We'll get Dr. Gregory Pearson settled into the studio, and he'll be back to talk about abnormal baby heads. That's coming up, right after this.

[Music]

Dr. Mike Patrick: Dr. Gregory Pearson is a plastic and reconstructive surgeon at Nationwide Children's Hospital and an assistant professor of Plastic Surgery at Ohio State University, College of Medicine. Dr. Pearson serves as director of the Center for Complex Craniofacial Disorders which is a multi-specialty clinic that cares for children with skull abnormalities, among other things.

That's what he's here to talk about today, skull problems or head problems in babies. Let's give a warm PediaCast welcome to Dr. Gregory Pearson. Thanks for stopping by today.

Dr. Gregory Pearson: Thank you for having me. I appreciate your time.

Dr. Mike Patrick: I appreciate you taking your time. I know plastic and reconstructive surgeons are busy. So I appreciate you stopping by and talking to us today.

I think a good place to start with this would just be a description of the make-up of the normal baby's skull and sort of how normal growth proceeds before we talk about what can go wrong.

0:08:22

Dr. Gregory Pearson: Great. A child's skull is made of multiple bony plates, and we call those places where the bony plates come together 'sutures' and where a couple bony plates come together as a 'soft spot' which everyone knows is also called a fontanelle. And those sutures kind of act as stretch which allow the skull to grow. Because the skull is actually is a passive player in this. The brain is what causes the skull to grow. And the skull grows because the brain pushes out. When you look at child's skull growth, there's tremendous brain growth in the first year of life. As a matter of fact, it triples in size and quadruples by the second year. And so, that's why it's very important to note, watch a child's skull in the early periods of time and monitor the head shape when they're in their first year of life.

Dr. Mike Patrick: You have this bone plates, and some of the names parents would be familiar with like the frontal bone is in the front, the temporal bone is kind of in the sides but towards the front.

We also have a lot of pediatric providers who listen to the show, too.

So , we have the parietal bones, which is a little further back from the temporal, and the occipital in the back. And then, you mentioned these sutures are between them. What are those made out of?

Dr. Gregory Pearson: They're just a physiological point where there is rapid deposition of bone and resorption of bone. The sutures, they're just where the junctions of the bones has come together. So they're not like an actual… You can feel them. You can actually palpate them and move them, where a normal bone, if you palpate a ridge, that can be a sign of a problem. But they're just a place which is very physiologically active for protein deposition and regulatory responses.

Dr. Mike Patrick: So the bones grow into those sutures and that's how the skull then gets healed?

Dr. Gregory Pearson: They grow from those sutures.

0:10:10

Dr. Mike Patrick: Okay.

Dr. Gregory Pearson: So the brain pushes and there's deposition along the sutures but it can also be some endocranial release of tissues and deposition on the outside as well.

Dr. Mike Patrick: So, eventually the bones are going to fuse together.

Dr. Gregory Pearson: Correct.

Dr. Mike Patrick: And once they do that, it's more difficult for the skull to grow?

Dr. Gregory Pearson: Correct.

Dr. Mike Patrick: So, if they do come together and fuse too soon and that's going to end up being a problem, which we'll talk about more as we move on.

So as we think about the skull growing and these plates and the important of the sutures, if everything's going correctly and there's not really a problem, should parents expect their baby's head to be perfectly round? Or are there going to be some what we call normal unevenness?

Dr. Gregory Pearson: There's typically some normal unevenness then. Most parents shouldn't expect their child's head to be perfectly round if you look at it, specifically, when you start talking about normal positioning changes that can affect the head shape, such as positional craniocephaly, which I know we're going to talk about in a little while as well. But, the idea that a child's head has to be perfectly round is not necessarily true and not necessarily what most kids' heads look like. A lot of kids' heads and end up looking like their parents' and if their parents' heads have some abnormal shapes, it can be kind of familial also.

Dr. Mike Patrick: So there are some genetic basis, too, for head shape in addition to environmental factors that we'll talk about.

A lot of times right after baby's born especially if it's a vaginal delivery, their head may have some unevenness just as it was forced through the small opening of the birth canal. So that shouldn't concern parents too, at least in the days after birth.

Dr. Gregory Pearson: Correct. Some kids will have some deformation from… There can be some deformation in the womb, for example if they were low-lying in the pelvis for the last several weeks, month of pregnancy, if they're up against their rib cage, if they're twin gestation. And some children, if it's a prolonged labor and a prolonged time in the canal, they can have some deformational changes to their head shape as well.

0:12:21

Additionally, some kids are born with it. You can have a little called a cephalohematoma where you have a little blood clot on the scalp which can cause some deformation as well.

And so, those things tend to round out with a little bit of time and tend to even out with a little bit of time. Kids with synostosis on the contrary tend to have a very different type of head shape and it tend to be more noticeable and it doesn't change with time. But most kids will have a little deformational changes that tend to get better over the first couple of weeks of life.

Dr. Mike Patrick: So by about two weeks of age, you'd expect it to sort of be rounding back out to what you'd expect other kids' heads to be looking like at that point. So after a couple of weeks, there's still something that's really appears uneven, it's time to take another into things.

Dr. Gregory Pearson: I would think so, yeah.

Dr. Mike Patrick: Okay. So how do physicians and also parents really evaluate head size and shape during those first couple of months of life.

Dr. Gregory Pearson: Yeah, great question. And really, it's a lot of clinical acumen. You need to measure the child's head circumference and make sure it's progressing along well with their growth curve. Every child will kind of establish their own growth curves. And every pediatrician's office has a chart of what normal should or shouldn't be based upon sex and age and things like that make-up.

The other thing is just to clinically evaluate them, kind of look at them from the top down. Look at their face, see if they have any frontal frontal bossing, get an idea how round their head is.

And look at their ears. I think their ears are really critical because ears can suggest what's going on. Because as changes happen within the skull base which is where the brain sits, the ears get moved at times as well. And so, have a look at it and see are they roundish? Are they're kind of triangular in the front, particularly in the forehead area?

Are they kind of long like a boat? Is one ear moved forward compared the other ear? And how does that compare to the flatness in the back of the head? Those are all the things you want to look at.

0:14:25

Dr. Mike Patrick: So, ears are important. You want to look and make sure that they're sort of even and that there's not… I mean, if you do see an unevenness in the ears, and that could be a concern that the skull is not growing properly.

Dr. Gregory Pearson: Well, it can be, depends from which way the ear moves. In the case of positional plagiocephaly, the ear frequently moves forward on the side of the flat spot. In the case of synostosis, they're actually get moved backward on the side of the flat spot. So we always tell parents that children with position plagiocephaly, their head look likes a parallelogram from up top. So I always, when I'm examining the children, I always have the parents put the child way down in the lab. And I stand above them and I look from the top down. I look at the ear position compared to the flatness.

Again, a parallelogram tends to suggest positional, and a trapezoid-type appearance when you look at the ears and I was on the front and the back tends to suggest synostosis.

Dr. Mike Patrick: And since parents don't have a lot of other baby heads to compare it to — but they do see a lot of babies — so they're noticing something just doesn't look quite right, that would definitely be something to bring up to the pediatrician. So they could be the one looking down from above and trying to figure out these shapes and what's normal and maybe not normal.

Dr. Gregory Pearson: Correct.

Dr. Mike Patrick: You talked about head circumference, so we measure that in centimeters or inches. But then as we think about those growth charts that reported as percentiles, explain what is a percentile. What does that mean?

Dr. Gregory Pearson: Percentile, they've basically taken thousands of kids' heads measurements and they have ideas of what the 50th percentile is in terms of how many kids at a certain age measure at that percentile versus 75%. And there's all these numbers that get plotted along their growth curve. It doesn't really matter necessarily what the number is more than your child is following his or her number.

0:16:21

Dr. Gregory Pearson: We see a lot of kids that have head circumferences at 25 and every time you plot them out, they're at 25th percentile. That's the important thing, is that they're following those natural growth curves that are established from a normative data.

Dr. Mike Patrick: So if you had, if you just took a group of a hundred of kids at random and lined them up with the number one having the smallest head circumference and number 100 having the largest, the percentile kind of give you the idea where your kid would fit in that line.

So if they're 50th percentile, they'd be right in the middle. Twenty-fifth, 24 kids would have smaller heads. And if they're at the 75th, 24 kids would have bigger heads.

So just a way to compare what we think is average. Of course, you have to assume then that that original sample was really representative of everyone.

This becomes important as we think about the first of our two abnormalities we're going to talk about — microcephaly, which is too small of a head and compared to what should be normal, and macrocephaly, which is too large. How then do we define those based on those percentiles?

Dr. Gregory Pearson: So, they're technically defined as two standard deviations away from the normative value. There's a lot of statistical talk and that doesn't mean a lot of things to a lot of people.

But basically, if you're graded on the 97th percentile, that's technically two standard deviations. That's considered macrocrania. And if you're less than two standard deviations which is the third percentile, lower than the third percentile, that's called microcephaly. And so, I think of the percentiles, not the standard deviation number, so higher than 97, macrocephaly, lower than 3, microcephaly.

Dr. Mike Patrick: So, these really are kids who are either the very bottom of the growth chart or at the very top of the growth chart. There's still a lot of wiggle room for what's normal as we consider microcephaly and macrocephaly.

0:18:08

Dr. Gregory Pearson: Precisely.

Dr. Mike Patrick: And then, in terms of what causes these things, I would imagine that there's still… I think the important thing is there's going to be those three out of every hundred kids who still are normal. They just have small heads and maybe it's just family trait?

Dr. Gregory Pearson: Correct. The most common thing, this particular thing macrocephaly is its familial. And if you're really concerned, you can measure the parents head. And it's a little hard sometimes to find a growth curve for some of the parents, but you could measure the parent's head and see if they have macrocephaly. Familial macrocephaly and even microcephaly is not that uncommon.

Of course, there's tons of syndromes associated with each of these things. With macrocephaly, you do have to worry about hydrocephalus which is a surgical issue because as the ventricles expand, if they're expanding too much — again, the skull grows because the brain pushes out — if the ventricles are getting bigger and bigger and bigger, it's going to cause macrocephaly.

The good thing about that is that's pretty easily diagnosed by an ultrasound. They can look at the ventricular size, particularly if the soft spot is still open.

And there's other metabolic disorders and syndromes like neurofibromatosis that can cause macrocephaly. But for the most part, there are a lot of kids who have benign macrocephaly they just inherited that way.

Dr. Mike Patrick: And this is why it's important that you're seeing a pediatrician who's learned on these things and is going to take into account the big picture. So, not only head size but also the rest of the physical examination where a child is, in terms of their development, to make a decision once they factor all of those things together.

I would also suspected that's reassuring when a kid sort of stays at a certain percentile as they growing.

Dr. Gregory Pearson: Precisely. I mean, the things that are more concerning to me are when a child passes or drops off of a curve chart, particularly when a child's been mildly macrocephalic, like churning along that 30th percentile. If they kind of stabilized and you see them couple of months later and their heads circumference really hasn't change, maybe see them a month or two later and hasn't changed. That's when things get really concerning.

0:20:19

Or, if a child was say at 85th percentile and all of a sudden, they bumped way up above the 97th percentile for unknown reason. Again, most children will establish their own kind of curve, and it's when you have a change off of that curve significantly that you start to really worry.

Dr. Mike Patrick: And there's a lots of things that can cause microcephaly or macrocephaly that also aren't necessary normal like we've talked about and in particular genetic disorders and syndromes and things. We'll kind of bypass by those because we could do an entire hour episode on microcephaly and then another hour on a macrocephaly. But just suffice it to say that it needs to be looked into. And it may not be anything. It may just be a family trait, but still you want to look into that more.

Dr. Gregory Pearson: It's something that should be explored. I agree that pediatricians should really be the first line of a knowledge base and monitoring for head shape and size and circumference and all those things that you spoke about.

Dr. Mike Patrick: Now, you've mentioned and I mentioned in the intro as well a big word, positional plagiocephaly. And I'm always interested in where these words come from. So, plagio is a Greek for slanting or unevenness. And cephaly, of course, is concerning, pertaining to the head. So this is really just a fancy name for a flat spot.

Dr. Gregory Pearson: Precisely. I mean, plagiocephaly when it was first described just mean an abnormal head shape. And it could mean you had synostosis, it could mean you had what's called positional plagiocephaly. Now and now, when people say plagiocephaly just in terms of conversation, it tends to be positional or something that's non-surgical. That's just kind of been the evolution of that term in terms of medical jargon in discussions.

And so, the plagiocephaly just means you have a slanted, flat head or an abnormal head shape. But it's not more descriptive than that.

0:22:15

Dr. Mike Patrick: And then, positional plagiocephaly, then we're starting to talk about maybe what causes that unevenness or that flat spot.

Dr. Gregory Pearson: Correct. In positional plagiocephaly, it's actually extremely common. Ever since the Back to Sleep Program, there's been a large uptick in positional plagiocephaly because of, again, as that molding pressure, as you lie on your back, you tend to mold the posterior aspects to flatten out.

It's been great for Sudden Infant Death Syndrome but ever since that, there's been a large increase. So much that some literature says that up about 46% of kids at six months of age, have some components of positional plagiocephaly.

Dr. Mike Patrick: We'd rather have a child who's alive, who has a flat spot on their head.

Dr. Gregory Pearson: Precisely.

Dr. Mike Patrick: Than the risk of death with Sudden Infant Death Syndrome lying on the belly.

Of course, I would imagine this is also pretty common in the neonatal intensive care unit where you have babies who are just lying there and not necessary being held as often as a normal baby because they're critically ill. So you see that a lot in the NICU.

Dr. Gregory Pearson: In the NICU, it's very common. It's common in kids who have severe torticollis because they can't arrange their neck as often. It's more common again in twin gestation, if the kid has a cervical spine issue. I've seen that occasionally where they have cervical stenosis and they can't rotate their head as much. That's not very common.

Most common in the scan is their back to sleep for the NICU where they're not up as much because they're critically ill and they can't get out of their crib from the safety standpoint.

Dr. Mike Patrick: Sure. And you mentioned torticollis, that's like a stiff muscle in the neck. And so, it'll be a kid who's maybe looking to one side all the time and so they're going to lie in the same position.

0:24:01

Dr. Gregory Pearson: Yeah, from just the tightness of the neck, they tend to prefer to have their head rotated in one way. And then it's once you start sleeping that way, I tell parents, you try when you're sleeping on your back, you trying to sleep on a ball. And once that ball gets a little bit flat, you always tend to play with that favorite flat spots.

Dr. Mike Patrick: Yeah, that totally makes sense. Now, does that affect the underlying brain?

Dr. Gregory Pearson: No, it doesn't. And that I think is probably the most important thing that you can tell parents. Positional plagiocephaly is just benign issue. It doesn't have any functional considerations. And you'd be amazed that the number of parents once they hear that, they're extremely relieved, because they're concerned about pressure on the child's brain. Or they're concerned about development. But positional plagiocephaly is benign, non-functional issue.

It just is a shape of his head but the skull is growing and the brain is developing normally. There was initially some concern that it might affect the vision and that's not been proven to be true. It doesn't affect cognition or anything like that. It's a towel around your head. And that's important. But once you get that functional component out of their heads, it's really reassuring.

Dr. Mike Patrick: Yeah, is this something that then as a child gets older and they're starting to have different sleep positions… So now they're not young infant anymore. It's not as important that they're on their back. So they're out of that range when we're worried about SIDS. Is this something that then it will round itself back out or does the flat spot stay?

Dr. Gregory Pearson: It kind of depends upon the severity of the flat spot, some other comorbidities or other things going on if the child has some what we call hypotonia, which means they're not quite lifting their head up as much. But a lot of times, especially in the more milder cases, it does get better, especially as you're sitting them up and holding them up and doing the 'boppy' or 'bamba' whatever it's called nowadays kind of thing.

And as they start to roll over in the crib, again you should always put your child back to sleep with nothing in the crib before a year of age. But some kids will start to roll from back to belly to sleep comfortably. So it does tend to round out.

0:26:05

Dr. Mike Patrick: I think this would be a good time to mention the safe sleep rules. And that's the ABCs — so alone and just the baby in the crib. Nothing else in there, just the baby, right?

Dr. Gregory Pearson: Correct.

Dr. Mike Patrick: Every single time.

Dr. Gregory Pearson: Every single time.

Dr. Mike Patrick: Yes. But as they do get older and they're moving around and in different sleep positions, if it's mild and this is something that could potentially correct on its own.

Dr. Gregory Pearson: Yeah, a lot of them do.

Dr. Mike Patrick: And then, sometimes you'll see babies wearing helmets for this. Is that something that is useful, not useful? How do you decide whether to do that or not?

Dr. Gregory Pearson: Some of it is based upon the severity. Because as a child has more severe positional plagiocephaly, they can actually have some facial asymmetry. So you kind of think about taking your hands that are right next to each, parallel to each other, and pushing one forward, you can see that your front would get affected as well. And that's why trying top down and look at the ear, because you tend to get a flat spot, and then the ear tends to move forward, and then the face tend s to move forward.

So for children who have some facial asymmetry, it can be very helpful, who have a pretty severe flat spot. Maybe they've tried conservative measures that we talked about. Helmets can be helpful. And sometimes, it's patient and parent preference and how nervous they are about how round their child's head shape needs to be.

Dr. Mike Patrick: So this is one of those issues where there's not always black-and-white, correct-or-wrong answer. You really just want to walk that journey with the family, let them know risk-benefits kind of thing.

Dr. Gregory Pearson: I tell my families that I can point to literature that is very strongly pro-helmet and very strongly anti-helmet. But you had to look at and see the bias in that literature and who is writing it, and whether they are big advocates of helmets or kind of anti-helmet. And so, I tell the families that you can pick the horse you want in the race based upon which literature you want to read.

Dr. Mike Patrick: Yeah. If it's a mild flat spot and the kid hates wearing the helmet, that's one situation. And on the other hand if the kids loves having the helmet on, then it's really not hurting anything to do.

What about costs? Are they expensive and does insurance typically pay for them?

0:28:13

Dr. Gregory Pearson: So they can be expensive. Insurance sometimes pays for them.

[Laughter]

Dr. Mike Patrick: Still not a black-and-white issue.

Dr. Gregory Pearson: It is not a black-and-white issue. Some insurance companies want to get… There are some measurements that can be done by the helmet companies where they measured the difference and they've tried to determine severity scores based on that. There's some debate over those numbers. And sometimes, it's considered something that's not required by insurance. It's not pre-existing condition but as an outlier because it does not have a functional component that's an exclusionary contract. So it's highly variable by insurance.

So it just becomes another one of those pieces of the puzzle as you're talking to the family about the advantages and disadvantages kind of thing.

Dr. Gregory Pearson: Precisely.

Dr. Mike Patrick: In kids who let's say though it is a fairly significant, there is some facial asymmetry, when it's significant like that, do those typically go away or is it then more likely to be an issue moving forward?

Dr. Gregory Pearson: The more severe it is, I think the more likelihood that it's going to stay in the… You may still have some facial asymmetry and that's… And I try to tell the parents, give them a pretty frank discussion about whether I think the helmet's going to be really helpful for them or not. And I think if you do have a lot of facial asymmetry, it's good because it does tend to help things out.

Dr. Mike Patrick: Yeah, it did totally make sense.

Dr. Mike Patrick: Let's move on to craniosynostosis. And again, let's break that word down. So cranio concerns the skull or the head. Syn, S-Y-N, is a prefix rooted in the idea or joining or fusing and ostosis refers to bone. So these are bone plates of the head in the areas where they are up against each other and where they will eventually fuse, is what we're talking about.

Dr. Gregory Pearson: Correct.

0:30:10

Dr. Mike Patrick: So let's talk first when should they close normally? What's normal for suture closure?

Dr. Gregory Pearson: Most of the sutures actually close in our 20s, which is surprising to most people. But when you look at them from studies, they close in 20s. With the exception of what's called the metopic suture. The metopic sutures run from the anterior fontanelle or the front soft spot down between the eyes.

And there are studies that show that suture actually closes around nine or ten months of age. There are the ones, the sagittal which goes from the anterior fontanelle or the anterior soft spot to the posterior fontanelle, posterior soft spot, closes in 20s.

The ones that go from the soft spot down by the ears called the coronal sutures, again 20s. And the lambdoids are in 20s, but not as active because, again, most of our brain growth happens early in life. But if you actually look at studies, they're still open.

Dr. Mike Patrick: So the brain itself is what cause that rapid growth especially in the first year of life, but we're still growing until we're in our early 20s. And our head has to grow to some degree, but it's just not as accelerated as it is early on.

So the soft spot itself though at some point goes away. You can't really feel it anymore. When should that happen? Because I know as a primary care provider, one of the things you do as you're feeling a child's head is to make sure that soft spot is still open. When would you expect that normally just to close?

Dr. Gregory Pearson: Usually, it's closed…

Dr. Mike Patrick: Or at least not there anymore.

Dr. Gregory Pearson: Yeah, not felt anymore by 24 months, 2 years of age. It's kind of the anterior spot is what we kind of tell people.

Dr. Mike Patrick: So if it's six months of age, you're not feeling it, that would raise some concern.

Dr. Gregory Pearson: It can be, yeah. And then, when you see a fair number of consults for children, their concern is the anterior fontanelle is closed too prematurely. And that's again one of those things where having good head circumference growth chart is very helpful. Because it might not palpable, but as long as it's still functional, that's the critical thing.

0:32:10

And if it closes too early and your head circumference is falling off the curve, that's concerning. If you can't feel it as much but you have a lot of data points that show that their head circumference has fallen down that 10th percentile whatever their percentile was, it shows that it's still functioning which is important to know.

Dr. Mike Patrick: So again, you got to look at the big picture rather than just one thing.

Dr. Gregory Pearson: Correct.

Dr. Mike Patrick: And when would you then call it craniosynostosis, that it's a problem?

Dr. Gregory Pearson: So craniosynostosis, again, as that premature fusion of those bones tends to really be noticeable at birth because most of them do fuse in the uterus. They can fuse shortly after birth. But most of them have a pretty classic presentation shortly after birth, and that depends upon which suture is fused.

So it's one of those things where we talked about you can have some deformation from the birth canal but the kids with craniosynostosis, particularly the frontal ones, the metopic, sutures or coronals sutures tend to have a pretty characteristic look to them. The metopic have a very triangular shaped-head and a lot of brow retrusion.

The coronals have some brow retrusion and a lot of times, you can even feel the ridging of these sutures. With the sagittal, it's being very long frontal back and kind of narrow on ear to ear.

They tend not to get better. They tend to always kind of look that way and it's just kind of typical hollow look.

Dr. Mike Patrick: And then, as you're looking at their head circumference, they're going to be more likely to be on the lower end?

Dr. Gregory Pearson: Can be. It depends on what sutures are fused. If it's sagittal sutures which goes from the from to the back, they can actually have bigger head shapes because they have expanded more rapidly in the front to back. So when you measure their circumference, they tend to actually have larger heads.

So having one or the other doesn't mean as much lot of the syndromic kids, particularly if you have more sutures closed, tend to have a more microcephalic feature. So it's really kind of suture dependent.

0:34:20

Dr. Mike Patrick: And, again, a reason that you want someone who's used to looking at kids day in and day out like a pediatrician taking care of your child right from the beginning.

So what causes this premature closure of sutures in kids that are affected?

Dr. Gregory Pearson: So when we talk about craniosynostosis, we kind of thought of it as idiopathic which means we either don't know or don't have any answer yet or kind of syndromic. That's the breakdown.

Again, the idiopathic just means it just happened. Why it's happening, we don't know realistically. We'll probably know more of these kids who have called idiopathic synostosis as genetic. Thus genes get better and as the whole genome project goes along and full exomal processing happens, we'll probably know more and these will get classified by mutations.

With the syndromic, they tend to have known mutations which can have synostosis particularly that can affect other areas of the body, the hands, the thumb, the toes and other things like that. So that's kind of the typical breakdown of the two.

Dr. Mike Patrick: Now, does that affect the brain?

Dr. Gregory Pearson: So craniosynostosis can affect the brain because unlike positional plagiocephaly where all the sutures are still open and the brain is just growing in response to gentle molding pressure, with craniosynostosis, the analogy that Dr. Governale, the neurosurgeon I work with, and I both make it's like that suture is now locked. And the brain can't expand against that locked suture. And so, certain percentage of kids can develop increased intracranial pressure, which is concerning.

0:36:01

Dr. Mike Patrick: Yeah, absolutely. And can it also affect brain development in terms of what kids are doing and are they doing the things that they ought to be at that particular age?

Dr. Gregory Pearson: So increased intracranial pressure is a big concern because it can affect development. It can affect sleeping and central apnea. It can affect speech and language. It can affect the motor gait. So it can really present a lot of developmental delays.

Dr. Mike Patrick: Sure. So how do you treat craniosynostosis once you discover it?

Dr. Gregory Pearson: So craniosynostosis is a surgical problem. You can't put a kid in a helmet and expect craniosynostosis to get better. Sometimes you use helmets after surgery to help mold the head. But you have to do something surgical to remove that synostosis suture. And that can be, depending upon the age of the child, something kind of minimally invasive with a helmet afterwards. Or sometimes, it's what's called an open cranial valve reconstruction.

Dr. Mike Patrick: So you really have to, where the suture has fused you want to open that back up.

Dr. Gregory Pearson: Yeah, we like to say we need to unlock that suture.

Dr. Mike Patrick: And the earlier that this is discovered and manage, the less invasive that treatment tends to be.

Dr. Gregory Pearson: Can be yeah. And we have a lot more options because you need for the earlier surgeries, the more minimally invasive surgeries, we're relying upon the rapid brain growth. And so for children that present later, sometimes we don't have as many options.

So yeah, the earlier we can diagnose it and see these families and treat these families, we have to just have more options to offer them based upon their preference, based upon the anatomy we're seeing and other factors.

Dr. Mike Patrick: Before the surgery, when you're really trying to characterize which sutures may be involved and as you're thinking about what approach and technique that you're going to use, is I would imagine you rely a lot on your physical exam. Are there some imaging studies that need to be done as well, and which ones are the best ones to use?

Dr. Gregory Pearson: So again, physical exam is the key, as you've stated. But in terms of imaging, CAT scan is really the best imaging for this. It used to be a skull series. The problem with skulls series is it's four series and sometimes, it can be non-diagnostic.

0:38:10

Fortunately, at Children's Hospital, we had the benefit of developing a low-dose CAT scan which shows the bone anatomy very well. It doesn't show the brain anatomy as much but that's not what we're looking for. And the radiation exposure is actually less than a typical of four-view skull series.

So not only is it more diagnostic and more… It actually got nice tweety pictures of their skull and what things look like, it's actually less radiation at the same time.

Dr. Mike Patrick: That's nice.

Dr. Gregory Pearson: So better information and less radiation.

Dr. Mike Patrick: MRI not as helpful with bones.

Dr. Gregory Pearson: MRI is not as helpful for the bones. If you're concerned about hydrocephalus or some underlying parynchemal or what that means they're actually crossed the brain soft tissue, the MRI is still preferred. But for bony abnormalities, we really prefer a CAT scan.

Dr. Mike Patrick: Ultrasounds, are they useful at all?

Dr. Gregory Pearson: Not really. Again, for the diagnosis of hydrocephalus, when the fontanelles are still open yes, but in terms of they can't look at their sutures and determine whether they're open or not.

Dr. Mike Patrick: And what happens if craniosynostosis goes untreated?

Dr. Gregory Pearson: So that's a big debate. Again, most of the kids, the risk is that they're going to develop that intracranial pressure. It's hard to know the exact percentage because in order to do that, you have to literally do what's called an endocranial pressure monitor which involves putting a child in the ICU for several days with monitors and invasive procedures.

So there's debate over what that exact number is, but left untreated, if a child does have intracranial pressure, they'll have delays. They can have vision loss because the optic nerves can have some compression on them, and frequent headaches, nausea, vomiting, central sleep apnea.

Dr. Mike Patrick: I think it is important to identify this and get it treated and managed appropriately in as early of an age as possible. Even with treatment though, you might still see some cognitive development delays. I would imagine, because if there are some underlying genetic syndromic kind of issues, that cause the cranial synostosis, then those might actually cause a problem with brain growth that's not even related to the sutures being fused prematurely. Would you agree with that?

0:40:26

Dr. Gregory Pearson: Oh, yeah, precisely, with the syndromic children because we haven't a lot of time to know actually what genetic mutation they have. They can have development delays and the synostosis may just represent the underlying genetic bias. Now, most kids with syndromic synostosis do tend to have normal growth and development. Some of these syndromes have a lower potential of IQ and things of that nature. But still, the reason to treat is to prevent that downslide or that back slip back into pressure and things of that nature.

Dr. Mike Patrick: I think one issue that becomes important for parents is if they are noticing that the head size is a little… They look at the head shape and they think, well, something's just not quite right here. At what point do you still just sort of watch things and when do you say, "Oh, no this is time now to refer and do something."

And the same thing for the primary care providers, too. What point do you watch it and what point do you say we need to do something? Are there some red flags that folks should be looking for?

Dr. Gregory Pearson: I think if you have some concerns early on, the primary care doctor I'm sure is going to see the child pretty frequently and measure that and look and clinically evaluate. Three months is a good time for us because it still gives us a lot of opportunities. And if you're concerned earlier, we're always happy to see the children earlier.

The challenge we get into is when it's been followed for eight or nine months and then that does limit some of our options.

Dr. Mike Patrick: So it makes some sense if it's kind of a soft call and you're not sure if there's really a problem, maybe wait till their next well checkups, like a month or two. And if it's still there though, it's time to refer, not to keep observing.

Dr. Gregory Pearson: Yeah.

Dr. Mike Patrick: But if you have significant concern even right from the beginning, it'd be better to refer and have someone take a look. Who should they refer to now?

0:42:29

So here in Central Ohio, here at Nationwide Children's, we have the Center for Complex Cranial Facial Disorders. And I understand they could refer to that clinic. But let's say other places in the country may not have a center like that. Is there a particular specialty that ought to see these kids?

Dr. Gregory Pearson: So in most tertiary hospitals, a plastic surgeon works with the neurosurgeon, both of them pediatric trained or craniofacial trained for the plastic surgeon. That's kind of the buzzword. And so, in most tertiary hospitals, those two work together very well. And in general, a referral to one will be a referral to the other.

Dr. Mike Patrick: Because they're going to consult one another and work as a team.

Dr. Gregory Pearson: Yes. Just like Dr. Governale and I, if he sees a patient who has synostosis, he will call me and will arrange a time for me to see them. A lot of times, we have the clinic on the same days, so we will cross-pollinate, spontaneous cross-fertilization, if that makes sense.

[Laughter]

Dr. Gregory Pearson: Just to make sure that we get the children in, get them seen promptly by both physicians, both talked to them about the diagnosis and how to proceed.

Dr. Mike Patrick: Should the primary care provider order the CT scan before they see you? So that is available for you to look at when you consult with the family or is that something that then your order when you see the child for the first time?

Dr. Gregory Pearson: I think it partially depends upon how far the family has to travel and some of those factors. If a family's traveling really far, I would say no, because somebody outside the hospital is A, don't have the technology to do the low-dose. And the great thing about the low-dose CAT scan is it's very quick. So a lot of times, we will just order them in the clinic.

0:44:08

If they're a little closer and it's not a hardship on the family and the pediatrician is in the Central Ohio area, if it doesn't hurt. But at the same time, if there's any concern because it involves radiation, just hold off and we can get it when we see the child, because we can get them in usually same day.

Dr. Mike Patrick: And after they have that then, do you talk to the family again? Or is that so they would go get the CT Scan and then come back to clinic? Or it kind of depends of what time of the day it is?

Dr. Gregory Pearson: A lot of times, the CT is just confirmatory and helps just in surgical planning, make sure that there's no other sutures that are closed that you didn't recognize or something like that. So a lot of times, we'll have them get the CAT scan, come back to clinic or maybe they'll get the CAT scan between seeing the two of us and things of that nature.

Dr. Mike Patrick: What other disorders does the Center for Complex Craniofacial Disorders, what other sort of things do you see in that clinic?

Dr. Gregory Pearson: So we see all sorts of children who had facial differences. And that can be children who have what's called hemifacial or craniofacial microsomia, which is one half of their face is not as developing as normally and that can be related to different factors in the womb. We see children who have microtia which is missing ears or portion of the ears didn't grow as correctly.

We see some acquired defects. There is the Parry-Romberg disease which is facial atrophy that can happen in teenage. We see children with fibrous dysplasia, which is a bony overgrowth. And then we see other children who have had post-traumatic defects and anything that really affects the facial skeleton.

Dr. Mike Patrick: Yeah, and facial paralysis, do you see much of that?

0:46:02

Dr. Gregory Pearson: I don't do that personally. One of my partners does have an interest in that. We see some of those kids and then make sure that they're seeing all the proper people for…

Dr. Mike Patrick: Yeah, like a Bell's palsy that's maybe not going away.

Dr. Gregory Pearson: The Bell's palsy, yeah.

Dr. Mike Patrick: And so, it's a multispecialty clinic. You mentioned neurosurgery and, of course, plastic and reconstructive surgery. What other disciplines are involved in the clinic?

Dr. Gregory Pearson: So we have ophthalmology, oculo-plastic surgeons that we consult with. We have pediatric ophthalmologist to monitor their vision and to perform fundoscopic evaluations for our kids with craniosynostosis.

For other deformities, we have dentists and orthodontists because a lot of these kids who have other craniofacial differences will require a dental work or orthodontic work. We have speech therapists because some kids with craniofacial microsomia can have asymmetrical point of their soft palate when they're speaking which can cause speech issues.

We have a psychologist — which is a wonderful thing because as kids get older, if they have craniofacial difference that can cause teasing and bullying — that offers support to both the child and the family a lot of times.

Dr. Mike Patrick: Yeah, absolutely. And those folks in also Social Work can help with all sorts of issues in terms of finances and school issues and all those in terms of finding resources. So great. So I love that multispecialty model and that folks can get all the services that they need right there in one place.

All right, well, Dr. Pearson. Thank you so much stopping by and talking to us about all the abnormalities that can take place with baby heads. We'll have lots of links in the Show Notes for folks. A link to the Center for Complex Craniofacial Disorders here at Nationwide Children's. We also have an information page on positional plagiocephaly and another one on craniosynostosis, both here from Nationwide Children's, we'll put links to those.

And then, in the intro to the program, I mentioned back in PediaCast 323, we did a whole episode on baby problems and craniosynostosis was one of the listener questions that we had answered. And so we talk a little a bit about that in that episode as well.

0:48:15

So Dr. Pearson, thanks so much for stopping by and talking with us today.

Dr. Gregory Pearson: My pleasure. Thank you for your time.

[Music]

Dr. Mike Patrick: All right, we are back with just enough time to say thanks to all of you for taking time out of your day and making PediaCast a part of it. Really do appreciate that.

Also, thanks to our guest, Dr. Gregory Pearson, Plastic and Reconstructive Surgeon and Director of the Center for Complex Craniofacial Disorders at Nationwide Children's Hospital. Really appreciate him taking time out of his busy day to join us and enlighten us on all sorts of things that cause abnormal baby heads.

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0:50:28

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All right, thanks again for stopping by, really appreciate it. And, until next time, this is Dr. Mike saying stay safe, stay healthy, and stay involved with your kids. So long, everybody.

[Music]

0:52:15

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