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Head Shape Anomalies in Babies
Lance Governale, MD, discusses head shape anomalies in babies He compares the clinical features of the different varieties of craniosynostosis. He differentiates craniosynostosis from positional plagiocephaly and he helps us to become familiar with the different treatment options for craniosynostosis and the need for early referral.
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Learn more about Lance Governale, MD
Lance Governale, MD
Dr. Governale obtained a Bachelor of Science in physiology and neurobiology from the University of Maryland, research training from the National Institutes of Health and a medical degree from Harvard Medical School. He then stayed at Harvard to complete neurosurgical residency training at Brigham and Women’s Hospital and pediatric neurosurgical fellowship training at Boston Children’s Hospital.Learn more about Lance Governale, MD
Transcription:
The University of Florida College of Medicine is accredited by the Accreditation Council for Continuing Medical Education, ACCME to provide continuing medical education for physicians. The University of Florida College of Medicine designates this enduring material for a maximum of .25 AMA PRA category 1 credit. Physicians should claim only the credit commensurate with the extent of their participation in this activity.
Melanie Cole (Host): Welcome to UF Health MedEd Cast with UF Health Shands Hospital. I’m Melanie Cole. And today, we’re discussing craniosynostosis and head shape anomalies in babies. Joining me is Dr. Lance Governale, he’s the Chief of Pediatric Neurosurgery at UF Health Shands Children’s Hospital, part of University of Florida in Gainesville, Florida. Dr. Governale, I’m so glad to have you join us today and what an interesting topic. Start by telling us what is craniosynostosis and what’s the prevalence? Tell us a little bit about it.
Lance Governale, MD (Guest): So, craniosynostosis is the early closure of at least one of the bone junctions, the sutures in a baby’s head. So, as most physicians know, the skull is made up by multiple different bones, the junctions in between which are called sutures. The sutures are what allow the skull to grow over time as the brain inside is trying to grow. Sometimes, one or more of these sutures can close early and that’s called craniosynostosis. It happens in about one in every about 2500 live births. When it closes, it can throw the head shape off in certain defined ways which can cause a deformity of the child’s head. But also, it can limit the overall head growth which becomes a risk for brain development over time and risk for pressure development. So, we do like to find it early and treat it early where possible.
Host: And I’m so glad you brought that up. It’s a great point because I’m going to ask you about the increased intracranial pressure and development. But before we do that, tell us a little bit about the different types. Compare the clinical features of the different varieties for us if you would.
Dr. Governale: Yeah, so each different suture can give a different head shape because what dictates the head shape is that when a suture closes, the skull can’t grow perpendicular to the suture and grows parallel instead. So, based on that, you can figure out the different shapes. But to go through them, the most common non-syndrome form of craniosynostosis is sagittal craniosynostosis. And when the sagittal suture closes, you get a long head that’s very narrow with prominence of the forehead and the occiput. That has a term called scaphocephaly. Some people get mired down in the terms but more importantly, is to recognize the shape. The opposite of that is when both coronal sutures close. You get a short wide head that sometimes can be very tall also. That’s called brachycephaly. And the tallness is called turricephaly. When the metopic suture closes early, you get a triangle shape to the forehead when you look at it from above called trigonocephaly. When one coronal suture or one of the lambdoid sutures closes in the back, you get a head shape that’s called plagiocephaly but it’s totally different from the positional deformational plagiocephaly that most people think of when that term comes about. Which is why again, I try not to get mired down in the terms but just mentioning them for completeness.
So, when one coronal suture closes, the forehead is flat on that side and you kind of get elevation of the orbital roof and a slanting of the nose. So, the face gets a kind of curved look to it. It looks like the child’s you an eye, in fact, sometimes people get referred for eyelid drooping ptosis on the other side when it’s actually the opposite side that has the issue. When one of the lambdoid sutures closes in the back, this is where you can get flattening in the back of the head similar to positional plagiocephaly but the difference and the key difference to determine in the office is that when you have positional plagiocephaly, the ear on the side of the flattening is pushed forward compared to the other side. However, if you have unilateral lambdoid craniosynostosis, the ear on the side of the flattening is pulled backwards. So, for positional plagiocephaly, you get a parallelogram head shape and for unilateral lambdoid craniosynostosis, you have a trapezoid shape to the head.
In addition, for the lambdoid craniosynostosis, when you look at the head from either the front or the back straight on as opposed to looking from the top; on the side of the flattening for craniosynostosis, the ear gets forced downward and you have a very prominent mastoid whereas for positional plagiocephaly, the ears stay level when looking at it from the front or the back. So, that’s an overview of the most common shape anomalies for the different sutures.
Host: That was a great overview, doctor. That was excellent and thank you so much for it. So, as long as we’re talking about all of these different varieties, tell us the possible complications and now is when you can bring up the effects on increased intracranial pressure or development. What are some of complications of this?
Dr. Governale: First off, the head shape deformity is a real issue both for the parents vie of their child but eventually, for the child’s view of themselves. So, that’s something we don’t want to discount and can become a social issue if not addressed early. Secondarily, like we talked about, there is the risk when a suture closes, that the skull won’t grow large enough for the brain inside that’s trying to grow. And that can lead to a size mismatch between brain and skull which can lead to pressure development in the head and increased intracranial pressure is not a good thing regardless of what causes it. So, because there is a risk of that developing over time, is another reason we like to treat craniosynostosis when we find it.
Now in terms of developmental affects, some of them can be related to increased intracranial pressure but there’s also evidence that you can have developmental issues from craniosynostosis even if you don’t have increased intracranial pressure just from the localized pressure. So, for all those reasons, we really want to find it early and treat it when we find it.
Host: So, when do you find it? When is it typically diagnosed?
Dr. Governale: So, it varies widely because it’s typically diagnosed by the pediatrician. So, that’s why these series of educational events are so important because it’s the recognition of the head shape and the referring physician that’s so key. So, with all the normal well checks, from the time of birth, it can be found. The sooner it’s found, the better. Now classically, pediatricians familiar with the diagnosis would also know that surgery typically wouldn’t happen until six months of age, so there’s some time to see if the head shape is going to correct. But these days, we have newer more minimally invasive surgery options which require earlier diagnosis and referral. So, for example, one of the options, the window to do the surgery is between about two and a half months and three and a half months of age and if it’s found after that, well the child is not a candidate for that type of minimally invasive surgery anymore so early diagnosis is key from the well checks at the time of birth.
Host: Well then let’s talk about the different treatment options and you mentioned the need for early referral and why that’s so important. Tell us some of the available options.
Dr. Governale: So, the classic treatment for craniosynostosis is the open surgery. So, the surgery that’s done at six months where we directly expose the locked bones because these closed sutures kind of locks the bones into position, we remove those bones in certain pieces which unlocks them and then we work to reshape them and put them back on. So, we are directly unlocking and reshaping the skull at the time of surgery. Those are the two goals for any of the types of surgeries to unlock the bones and reshape the skull. By the way, all of this is done with our pediatric craniofacial plastic surgeon. We work as one big team. Her name is Jessica Ching at our institution. Which is a key point to these surgeries.
Now in terms of more minimally invasive options that we have these days, there’s one option where we at surgery we excise the fused suture, about a 1.5 centimeter strip of bone including the fused suture and we do that through either one or two small about one inch incisions whether it’s one or two depends on the suture that has closed. By excising that strip of bone, we’ve unlocked the suture. Now we haven’t done the reshaping at the time of surgery. So, after surgery, to do the reshaping, we use a helmet, so a special helmet that’s sometimes used for the positional plagiocephaly variant but here specifically targeted for the postop craniosynostosis patient that then redirects the skull growth over time to reshape the head over time. Now we usually use that helmet until about the first birthday. And this is the surgery that has the window of two and a half to three and a half months of age.
We also have another minimally invasive option here where we, instead of a helmet, we use a spring assist option. So, this surgery is done about between three and six months of age, so it give us a little more time window to offer minimally invasive options here at UF. And that surgery also involves similar, we excise a 1.5 centimeter strip of bone. The incision is about an inch or two, still very small incision. We usually do one incision for this surgery. Then after the bone is unlocked, we put in two springs to provide a distractive force outward to keep the suture open and not closing too early and give us the dimension we want. Now the springs are not coil springs, they are crescent springs so, they lie nice and flat under the skin and keep the bone distracted. Now we do have to go back to surgery about four months later to remove those. But that’s through the same incision. It’s an even smaller surgery and people either go home the same day or the next day.
For both minimally invasive options, people usually stay in the hospital overnight and look pretty good the next day and they are usually ready to go home.
Host: Wow. So, so many treatment options Doctor. Does a child with craniosynostosis require frequent medical evaluations and follow ups? Speak about the follow up a little bit to ensure that the skull, facial bones, that everything is developing normally. What does that look like? What’s involved in the long term monitoring of this pediatric condition?
Dr. Governale: So, key of the long term monitoring is multidisciplinary follow up with a multidisciplinary team like we have at UF. I’ve mentioned our pediatric craniofacial plastic surgeon Jessica Ching who I work hand in hand with. We both then work with a whole series of people at our multidisciplinary craniosynostosis clinic to follow people over time. Because no additional issues may develop, but if they do, we want to have the people there to follow them and jump right on the issue and take care of it including therapists and audiologists and ENT doctors and dentists and every other type of physician these children could potentially need.
And we follow them for a long time. So, even if we do surgery at between three to six months, we initially follow close but once things settle, we do continue to follow annually to at least six to seven years of age when your about close to your adult head size, believe it or not. But the multidisciplinary follow up is very important.
Host: Well thank you for telling us about that approach. It is, as you say, so important. What’s included in patient education for pediatric craniosynostosis Doctor? So how do you work with families and tell us a little bit more about how the multidisciplinary team helps the families to go through what can be a pretty stressful time?
Dr. Governale: Any time we do a new evaluation of a patient for potential craniosynostosis, we get it that people are very scared and fearful. I mean as soon as your pediatrician mentions the word neurosurgeon, the fear is honestly going to kick in instantly and we have a – we all have children, so we understand that. I tell people most of my job is reassurance. Yes, for people who really do have craniosynostosis, I have to do surgery anyway, but number one we have to educate and reassure the family and we go through a whole education presentation in the office and in fact, I use the same slides I use when I give talks on the subject to other physicians with the family, just take them through it step by step and demystify the condition and the diagnosis and what we’re going to do and remove all the scary terms.
I mean even the term craniosynostosis is very scary if you haven’t heard of it. So, that is very important as we go through everything. And then, the repetition and foundation becomes a factor too, so I meet with everybody and then sometimes I meet with them initially with Dr. Ching but sometimes I’ll see them myself and then Dr. Ching will see them also and we get a chance to go through it again. We give the family another chance after they’ve kind of digested everything the first pass to talk about it again where needed.
And the multidisciplinary team like you said, it’s all about educating everybody for it. included in the multidisciplinary team, we do have psychologists and social workers et cetera, that can help with more direct issues with adjustment to the diagnosis and treatment.
Host: As we wrap up, Dr. Governale, please reiterate the importance of the need for early referral because that is truly important in this case and wrap it up with the take home message for other providers.
Dr. Governale: These days, with optimal craniosynostosis management, multiple different things are important. Early diagnosis and referral allows us to offer minimally invasive options that previously weren’t offerable. Secondarily, referral to an institution with a bonafide multidisciplinary craniofacial team is critical. And in addition, referral to an institution that has ultralow dose imaging and minimally invasive surgery options is key.
Host: Thank you so much Doctor, for joining us today and sharing your incredible expertise. To lean more about pediatric craniosynostosis please visit www.ufhealth.org/pediatricneurosurgery. Or to refer your patient please visit www.ufhealth.org/medmatters to get connected with one of our providers. And that concludes today’s episode of UF Health MedEd Cast with UF Health Shands Hospital. Please remember to subscribe, rate and review this podcast and all the other UF Health Shands Hospital podcasts. I’m Melanie Cole.
The University of Florida College of Medicine is accredited by the Accreditation Council for Continuing Medical Education, ACCME to provide continuing medical education for physicians. The University of Florida College of Medicine designates this enduring material for a maximum of .25 AMA PRA category 1 credit. Physicians should claim only the credit commensurate with the extent of their participation in this activity.
Melanie Cole (Host): Welcome to UF Health MedEd Cast with UF Health Shands Hospital. I’m Melanie Cole. And today, we’re discussing craniosynostosis and head shape anomalies in babies. Joining me is Dr. Lance Governale, he’s the Chief of Pediatric Neurosurgery at UF Health Shands Children’s Hospital, part of University of Florida in Gainesville, Florida. Dr. Governale, I’m so glad to have you join us today and what an interesting topic. Start by telling us what is craniosynostosis and what’s the prevalence? Tell us a little bit about it.
Lance Governale, MD (Guest): So, craniosynostosis is the early closure of at least one of the bone junctions, the sutures in a baby’s head. So, as most physicians know, the skull is made up by multiple different bones, the junctions in between which are called sutures. The sutures are what allow the skull to grow over time as the brain inside is trying to grow. Sometimes, one or more of these sutures can close early and that’s called craniosynostosis. It happens in about one in every about 2500 live births. When it closes, it can throw the head shape off in certain defined ways which can cause a deformity of the child’s head. But also, it can limit the overall head growth which becomes a risk for brain development over time and risk for pressure development. So, we do like to find it early and treat it early where possible.
Host: And I’m so glad you brought that up. It’s a great point because I’m going to ask you about the increased intracranial pressure and development. But before we do that, tell us a little bit about the different types. Compare the clinical features of the different varieties for us if you would.
Dr. Governale: Yeah, so each different suture can give a different head shape because what dictates the head shape is that when a suture closes, the skull can’t grow perpendicular to the suture and grows parallel instead. So, based on that, you can figure out the different shapes. But to go through them, the most common non-syndrome form of craniosynostosis is sagittal craniosynostosis. And when the sagittal suture closes, you get a long head that’s very narrow with prominence of the forehead and the occiput. That has a term called scaphocephaly. Some people get mired down in the terms but more importantly, is to recognize the shape. The opposite of that is when both coronal sutures close. You get a short wide head that sometimes can be very tall also. That’s called brachycephaly. And the tallness is called turricephaly. When the metopic suture closes early, you get a triangle shape to the forehead when you look at it from above called trigonocephaly. When one coronal suture or one of the lambdoid sutures closes in the back, you get a head shape that’s called plagiocephaly but it’s totally different from the positional deformational plagiocephaly that most people think of when that term comes about. Which is why again, I try not to get mired down in the terms but just mentioning them for completeness.
So, when one coronal suture closes, the forehead is flat on that side and you kind of get elevation of the orbital roof and a slanting of the nose. So, the face gets a kind of curved look to it. It looks like the child’s you an eye, in fact, sometimes people get referred for eyelid drooping ptosis on the other side when it’s actually the opposite side that has the issue. When one of the lambdoid sutures closes in the back, this is where you can get flattening in the back of the head similar to positional plagiocephaly but the difference and the key difference to determine in the office is that when you have positional plagiocephaly, the ear on the side of the flattening is pushed forward compared to the other side. However, if you have unilateral lambdoid craniosynostosis, the ear on the side of the flattening is pulled backwards. So, for positional plagiocephaly, you get a parallelogram head shape and for unilateral lambdoid craniosynostosis, you have a trapezoid shape to the head.
In addition, for the lambdoid craniosynostosis, when you look at the head from either the front or the back straight on as opposed to looking from the top; on the side of the flattening for craniosynostosis, the ear gets forced downward and you have a very prominent mastoid whereas for positional plagiocephaly, the ears stay level when looking at it from the front or the back. So, that’s an overview of the most common shape anomalies for the different sutures.
Host: That was a great overview, doctor. That was excellent and thank you so much for it. So, as long as we’re talking about all of these different varieties, tell us the possible complications and now is when you can bring up the effects on increased intracranial pressure or development. What are some of complications of this?
Dr. Governale: First off, the head shape deformity is a real issue both for the parents vie of their child but eventually, for the child’s view of themselves. So, that’s something we don’t want to discount and can become a social issue if not addressed early. Secondarily, like we talked about, there is the risk when a suture closes, that the skull won’t grow large enough for the brain inside that’s trying to grow. And that can lead to a size mismatch between brain and skull which can lead to pressure development in the head and increased intracranial pressure is not a good thing regardless of what causes it. So, because there is a risk of that developing over time, is another reason we like to treat craniosynostosis when we find it.
Now in terms of developmental affects, some of them can be related to increased intracranial pressure but there’s also evidence that you can have developmental issues from craniosynostosis even if you don’t have increased intracranial pressure just from the localized pressure. So, for all those reasons, we really want to find it early and treat it when we find it.
Host: So, when do you find it? When is it typically diagnosed?
Dr. Governale: So, it varies widely because it’s typically diagnosed by the pediatrician. So, that’s why these series of educational events are so important because it’s the recognition of the head shape and the referring physician that’s so key. So, with all the normal well checks, from the time of birth, it can be found. The sooner it’s found, the better. Now classically, pediatricians familiar with the diagnosis would also know that surgery typically wouldn’t happen until six months of age, so there’s some time to see if the head shape is going to correct. But these days, we have newer more minimally invasive surgery options which require earlier diagnosis and referral. So, for example, one of the options, the window to do the surgery is between about two and a half months and three and a half months of age and if it’s found after that, well the child is not a candidate for that type of minimally invasive surgery anymore so early diagnosis is key from the well checks at the time of birth.
Host: Well then let’s talk about the different treatment options and you mentioned the need for early referral and why that’s so important. Tell us some of the available options.
Dr. Governale: So, the classic treatment for craniosynostosis is the open surgery. So, the surgery that’s done at six months where we directly expose the locked bones because these closed sutures kind of locks the bones into position, we remove those bones in certain pieces which unlocks them and then we work to reshape them and put them back on. So, we are directly unlocking and reshaping the skull at the time of surgery. Those are the two goals for any of the types of surgeries to unlock the bones and reshape the skull. By the way, all of this is done with our pediatric craniofacial plastic surgeon. We work as one big team. Her name is Jessica Ching at our institution. Which is a key point to these surgeries.
Now in terms of more minimally invasive options that we have these days, there’s one option where we at surgery we excise the fused suture, about a 1.5 centimeter strip of bone including the fused suture and we do that through either one or two small about one inch incisions whether it’s one or two depends on the suture that has closed. By excising that strip of bone, we’ve unlocked the suture. Now we haven’t done the reshaping at the time of surgery. So, after surgery, to do the reshaping, we use a helmet, so a special helmet that’s sometimes used for the positional plagiocephaly variant but here specifically targeted for the postop craniosynostosis patient that then redirects the skull growth over time to reshape the head over time. Now we usually use that helmet until about the first birthday. And this is the surgery that has the window of two and a half to three and a half months of age.
We also have another minimally invasive option here where we, instead of a helmet, we use a spring assist option. So, this surgery is done about between three and six months of age, so it give us a little more time window to offer minimally invasive options here at UF. And that surgery also involves similar, we excise a 1.5 centimeter strip of bone. The incision is about an inch or two, still very small incision. We usually do one incision for this surgery. Then after the bone is unlocked, we put in two springs to provide a distractive force outward to keep the suture open and not closing too early and give us the dimension we want. Now the springs are not coil springs, they are crescent springs so, they lie nice and flat under the skin and keep the bone distracted. Now we do have to go back to surgery about four months later to remove those. But that’s through the same incision. It’s an even smaller surgery and people either go home the same day or the next day.
For both minimally invasive options, people usually stay in the hospital overnight and look pretty good the next day and they are usually ready to go home.
Host: Wow. So, so many treatment options Doctor. Does a child with craniosynostosis require frequent medical evaluations and follow ups? Speak about the follow up a little bit to ensure that the skull, facial bones, that everything is developing normally. What does that look like? What’s involved in the long term monitoring of this pediatric condition?
Dr. Governale: So, key of the long term monitoring is multidisciplinary follow up with a multidisciplinary team like we have at UF. I’ve mentioned our pediatric craniofacial plastic surgeon Jessica Ching who I work hand in hand with. We both then work with a whole series of people at our multidisciplinary craniosynostosis clinic to follow people over time. Because no additional issues may develop, but if they do, we want to have the people there to follow them and jump right on the issue and take care of it including therapists and audiologists and ENT doctors and dentists and every other type of physician these children could potentially need.
And we follow them for a long time. So, even if we do surgery at between three to six months, we initially follow close but once things settle, we do continue to follow annually to at least six to seven years of age when your about close to your adult head size, believe it or not. But the multidisciplinary follow up is very important.
Host: Well thank you for telling us about that approach. It is, as you say, so important. What’s included in patient education for pediatric craniosynostosis Doctor? So how do you work with families and tell us a little bit more about how the multidisciplinary team helps the families to go through what can be a pretty stressful time?
Dr. Governale: Any time we do a new evaluation of a patient for potential craniosynostosis, we get it that people are very scared and fearful. I mean as soon as your pediatrician mentions the word neurosurgeon, the fear is honestly going to kick in instantly and we have a – we all have children, so we understand that. I tell people most of my job is reassurance. Yes, for people who really do have craniosynostosis, I have to do surgery anyway, but number one we have to educate and reassure the family and we go through a whole education presentation in the office and in fact, I use the same slides I use when I give talks on the subject to other physicians with the family, just take them through it step by step and demystify the condition and the diagnosis and what we’re going to do and remove all the scary terms.
I mean even the term craniosynostosis is very scary if you haven’t heard of it. So, that is very important as we go through everything. And then, the repetition and foundation becomes a factor too, so I meet with everybody and then sometimes I meet with them initially with Dr. Ching but sometimes I’ll see them myself and then Dr. Ching will see them also and we get a chance to go through it again. We give the family another chance after they’ve kind of digested everything the first pass to talk about it again where needed.
And the multidisciplinary team like you said, it’s all about educating everybody for it. included in the multidisciplinary team, we do have psychologists and social workers et cetera, that can help with more direct issues with adjustment to the diagnosis and treatment.
Host: As we wrap up, Dr. Governale, please reiterate the importance of the need for early referral because that is truly important in this case and wrap it up with the take home message for other providers.
Dr. Governale: These days, with optimal craniosynostosis management, multiple different things are important. Early diagnosis and referral allows us to offer minimally invasive options that previously weren’t offerable. Secondarily, referral to an institution with a bonafide multidisciplinary craniofacial team is critical. And in addition, referral to an institution that has ultralow dose imaging and minimally invasive surgery options is key.
Host: Thank you so much Doctor, for joining us today and sharing your incredible expertise. To lean more about pediatric craniosynostosis please visit www.ufhealth.org/pediatricneurosurgery. Or to refer your patient please visit www.ufhealth.org/medmatters to get connected with one of our providers. And that concludes today’s episode of UF Health MedEd Cast with UF Health Shands Hospital. Please remember to subscribe, rate and review this podcast and all the other UF Health Shands Hospital podcasts. I’m Melanie Cole.