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Craniosynostosis in Babies: Diagnosis, Treatment and Prognosis
Sometimes the bones of a baby's skull join together too early in a condition called craniosynostosis. Dr. Paul Klimo, chief of Pediatric Neurosurgery at Le Bonheur, discusses the signs and treatments of this relatively common condition.
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Learn more about Paul Klimo, MD
Paul Klimo, MD
Paul Klimo, MD is the Chief of the Division of Pediatric Neurosurgery and Associate Professor, The University of Tennessee Health Science Center.Learn more about Paul Klimo, MD
Transcription:
Craniosynostosis in Babies: Diagnosis, Treatment and Prognosis
Bill Klaproth (host): Many newborns and babies may have flat spots or an uneven head shape. Sometimes this isn't a cause for concern, but sometimes it is, and it's called craniosynostosis. So when is it a cause for concern and what is it? Well, let's find out what Dr. Paul Klemow.
Co-director of the neuroscience Institute and chief of pediatric neurosurgery at Lubana children's hospital.
This is the Peds Pod by Le Bonheur Children's Hospital. I'm Bill Klaproth. Dr Klimo, thank you so much for your time. So, let's get right into this. Can you explain to us what is craniosynostosis?
Dr. Paul Klimo: Craniosynostosis is where one or more of the sutures in the baby's skull prematurely closes and that results in restricted head growth and, therefore, an abnormal shape to the baby's head.
Bill Klaproth (host): And how common is this?
Dr. Paul Klimo: It's about one in 2,000 to one in 2,500 births. We see probably about 40 to 50 per year.
Bill Klaproth (host): Okay. Got it. So, I'm sure a parent has this question, is there any way to tell if my child has craniosynostosis in utero?
Dr. Paul Klimo: In utero, it would probably be a bit challenging. Currently, in utero imaging would be either an ultrasound or an MRI. And since craniosynostosis is a manifestation of early closure of bone, MRI may be a little bit limited, but you could maybe get a sense of the overall head shape on an MRI. Ultrasound, I think, would be a little bit more challenging, but possible. Usually, the diagnosis is made after birth.
Bill Klaproth (host): Okay. So usually, the diagnosis is made after birth. How would a parent know or suspect that their baby has craniosynostosis? Are there symptoms?
Dr. Paul Klimo: There's typically not symptoms. How most kids present to us is either the parents will notice that, at birth or shortly after birth, they felt that the baby's head was not normal in the shape. The other way is with the pediatrician also having a concern about the head shape sometimes. There is a ridge along the suture, the growth plate that has prematurely closed. Depending on which growth plate has closed, the head will grow in an abnormal shape. So some heads will be long and narrow. Sometimes there can be a flattening on the forehead and around where the eye socket is. So it all depends on which suture has prematurely closed. But typically, it's either parents feel "My baby's head shape is just a little bit abnormal," especially compared to, say, siblings or the pediatrician will have a concern.
Bill Klaproth (host): Right. So is this something that happens generally, like weeks after birth or months after birth? When is this usually discovered?
Dr. Paul Klimo: Both. I mean, sometimes we see them early when there is clearly an abnormal head shape. Sometimes the head shape can be a little bit abnormal shortly after birth, just from the birthing process itself. So, sometimes babies can have a little bit of a swollen scalp, swollen head. So therefore, the diagnosis may get pushed back by a few months. So usually, I would say most of the kids that we see with craniosynostosis are diagnosed within the first, say, three to six months of life.
Bill Klaproth (host): And if a parent suspects this, they should make an appointment right away. Is that right?
Dr. Paul Klimo: They should bring their concern probably to the pediatrician and then be evaluated. Yet, a lot of what is thought to be craniosynostosis is actually just what we call a positional plagiocephaly, which basically is some flattening of the child's head simply because of positioning. There's a push and there has been for the last number of years to keep babies sleeping on their back, at least for the first few months of life. And that results in some kids having, you know, an area of their head that gets a little bit flat. And so that flattening can sometimes be misinterpreted or it's diagnosed as craniosynostosis.
Bill Klaproth (host): Right. So if a diagnosis is made for craniosynostosis, what are the treatments available?
Dr. Paul Klimo: Craniosynostosis typically will not get better unless corrective surgery is performed. And there are several types of surgeries that can be done depending on the age and the type of craniosynostosis, but it is usually a surgical disease.
Bill Klaproth (host): And at what age is the surgery usually done? Is it right away? Does the baby have to get older before this happens?
Dr. Paul Klimo: So it depends. And surgeons have their preferences and their own opinions and philosophies about surgery. If the child is diagnosed young, so for example, within the first few months of life, they may be a candidate for what's called an endoscopic surgery, which is basically where you make a couple of small incisions at the front and backend of a few suture and you just simply take the suture out. And then, you have to treat that baby with a molding helmet for probably a good 10 to 12 months. The reshaping of the head is really done by the helmet and the growth of the baby's brain. If the child is a little bit older or if their craniosynostosis is more complicated, for example, in kids with what we call syndromic craniosynostosis, so they have some kind of genetic inborn syndrome that predisposes them to having craniosynostosis, those kids typically require more of a traditional open approach where you make a larger incision, larger cuts in the bone, but then the child does not need to be put in a molding helmet after surgery. So it really depends on age and complexity of the craniosynostosis.
Bill Klaproth (host): So then, what is the long-term prognosis for a child with craniosynostosis? How are some of your patients doing today?
Dr. Paul Klimo: In general, they do great. It's typically a one and done surgery. Certainly, for those that are the more simple single suture craniosynostosis. And by that, I mean just one suture has been involved. There's no syndrome. There's no multiple sutures being involved. Those children typically have a great long-term result. One operation and everything is corrected.
The ones that are more complicated, where there are multiple sutures involved or those children that have certain syndromes like Apert syndrome, Crouzon syndrome. Those kids have a higher risk of needing further surgery because sometimes, when you do the original or the initial correction, the sutures can re-fuse. And so you need to redo it again down the road. But those kids are more complicated.
Bill Klaproth (host): Right. How do the babies normally handle this surgery?
Dr. Paul Klimo: Typically very well. Sometimes they need a blood transfusion. Probably more common in those kids that have the open approach, where you're making a bigger skin incision, making more cuts in the bone. But otherwise, they do great. For the open approach, those children are probably in the hospital for maybe few extra days compared to the endoscopic approach. But in general, babies do very well from the surgery.
Bill Klaproth (host): So I'm sure for parents, this can be a scary time. What would you say to parents who have just received a diagnosis of craniosynostosis and are anxious about their child's health?
Dr. Paul Klimo: Sure. So I walk them through it and tell them exactly what's going on. Usually, I'm very upfront that the child needs corrective surgery. But in general, it's a very straightforward, very safe operation. The surgery is typically done in conjunction with my plastic surgery partner. It's always a two-part operation in the sense that I, as the neurosurgeon, am usually the one making the cuts in the bone and taking the bone off; plastic surgery is the one that typically reshapes and puts the bones back together. It's very well tolerated in kids. And it has an excellent success rate. Children tolerate it very, very well. So that usually takes care of a lot of the anxiety.
Bill Klaproth (host): Absolutely. Well, Dr. Klimo, thank you so much for your time. This is really interesting and fascinating, and you've been very informative. So again, thank you for your time. We appreciate it.
Dr. Paul Klimo: You're very welcome.
Bill Klaproth (host): And once again, this is Dr. Paul Kilmo. And to learn more about neurosurgery at Le Bonheur, please visit lebonheur.org/neuroscience. And be sure to subscribe to the Peds Pod on Apple Podcasts, Google Podcasts, or wherever you listen to your podcasts. You can also check out lebonheur.org/podcast to view our full podcast library. And if you found this podcast helpful, please share it on your social channels. This is the Peds Pod by Le Bonheur Children's Hospital. Thanks for listening.
Craniosynostosis in Babies: Diagnosis, Treatment and Prognosis
Bill Klaproth (host): Many newborns and babies may have flat spots or an uneven head shape. Sometimes this isn't a cause for concern, but sometimes it is, and it's called craniosynostosis. So when is it a cause for concern and what is it? Well, let's find out what Dr. Paul Klemow.
Co-director of the neuroscience Institute and chief of pediatric neurosurgery at Lubana children's hospital.
This is the Peds Pod by Le Bonheur Children's Hospital. I'm Bill Klaproth. Dr Klimo, thank you so much for your time. So, let's get right into this. Can you explain to us what is craniosynostosis?
Dr. Paul Klimo: Craniosynostosis is where one or more of the sutures in the baby's skull prematurely closes and that results in restricted head growth and, therefore, an abnormal shape to the baby's head.
Bill Klaproth (host): And how common is this?
Dr. Paul Klimo: It's about one in 2,000 to one in 2,500 births. We see probably about 40 to 50 per year.
Bill Klaproth (host): Okay. Got it. So, I'm sure a parent has this question, is there any way to tell if my child has craniosynostosis in utero?
Dr. Paul Klimo: In utero, it would probably be a bit challenging. Currently, in utero imaging would be either an ultrasound or an MRI. And since craniosynostosis is a manifestation of early closure of bone, MRI may be a little bit limited, but you could maybe get a sense of the overall head shape on an MRI. Ultrasound, I think, would be a little bit more challenging, but possible. Usually, the diagnosis is made after birth.
Bill Klaproth (host): Okay. So usually, the diagnosis is made after birth. How would a parent know or suspect that their baby has craniosynostosis? Are there symptoms?
Dr. Paul Klimo: There's typically not symptoms. How most kids present to us is either the parents will notice that, at birth or shortly after birth, they felt that the baby's head was not normal in the shape. The other way is with the pediatrician also having a concern about the head shape sometimes. There is a ridge along the suture, the growth plate that has prematurely closed. Depending on which growth plate has closed, the head will grow in an abnormal shape. So some heads will be long and narrow. Sometimes there can be a flattening on the forehead and around where the eye socket is. So it all depends on which suture has prematurely closed. But typically, it's either parents feel "My baby's head shape is just a little bit abnormal," especially compared to, say, siblings or the pediatrician will have a concern.
Bill Klaproth (host): Right. So is this something that happens generally, like weeks after birth or months after birth? When is this usually discovered?
Dr. Paul Klimo: Both. I mean, sometimes we see them early when there is clearly an abnormal head shape. Sometimes the head shape can be a little bit abnormal shortly after birth, just from the birthing process itself. So, sometimes babies can have a little bit of a swollen scalp, swollen head. So therefore, the diagnosis may get pushed back by a few months. So usually, I would say most of the kids that we see with craniosynostosis are diagnosed within the first, say, three to six months of life.
Bill Klaproth (host): And if a parent suspects this, they should make an appointment right away. Is that right?
Dr. Paul Klimo: They should bring their concern probably to the pediatrician and then be evaluated. Yet, a lot of what is thought to be craniosynostosis is actually just what we call a positional plagiocephaly, which basically is some flattening of the child's head simply because of positioning. There's a push and there has been for the last number of years to keep babies sleeping on their back, at least for the first few months of life. And that results in some kids having, you know, an area of their head that gets a little bit flat. And so that flattening can sometimes be misinterpreted or it's diagnosed as craniosynostosis.
Bill Klaproth (host): Right. So if a diagnosis is made for craniosynostosis, what are the treatments available?
Dr. Paul Klimo: Craniosynostosis typically will not get better unless corrective surgery is performed. And there are several types of surgeries that can be done depending on the age and the type of craniosynostosis, but it is usually a surgical disease.
Bill Klaproth (host): And at what age is the surgery usually done? Is it right away? Does the baby have to get older before this happens?
Dr. Paul Klimo: So it depends. And surgeons have their preferences and their own opinions and philosophies about surgery. If the child is diagnosed young, so for example, within the first few months of life, they may be a candidate for what's called an endoscopic surgery, which is basically where you make a couple of small incisions at the front and backend of a few suture and you just simply take the suture out. And then, you have to treat that baby with a molding helmet for probably a good 10 to 12 months. The reshaping of the head is really done by the helmet and the growth of the baby's brain. If the child is a little bit older or if their craniosynostosis is more complicated, for example, in kids with what we call syndromic craniosynostosis, so they have some kind of genetic inborn syndrome that predisposes them to having craniosynostosis, those kids typically require more of a traditional open approach where you make a larger incision, larger cuts in the bone, but then the child does not need to be put in a molding helmet after surgery. So it really depends on age and complexity of the craniosynostosis.
Bill Klaproth (host): So then, what is the long-term prognosis for a child with craniosynostosis? How are some of your patients doing today?
Dr. Paul Klimo: In general, they do great. It's typically a one and done surgery. Certainly, for those that are the more simple single suture craniosynostosis. And by that, I mean just one suture has been involved. There's no syndrome. There's no multiple sutures being involved. Those children typically have a great long-term result. One operation and everything is corrected.
The ones that are more complicated, where there are multiple sutures involved or those children that have certain syndromes like Apert syndrome, Crouzon syndrome. Those kids have a higher risk of needing further surgery because sometimes, when you do the original or the initial correction, the sutures can re-fuse. And so you need to redo it again down the road. But those kids are more complicated.
Bill Klaproth (host): Right. How do the babies normally handle this surgery?
Dr. Paul Klimo: Typically very well. Sometimes they need a blood transfusion. Probably more common in those kids that have the open approach, where you're making a bigger skin incision, making more cuts in the bone. But otherwise, they do great. For the open approach, those children are probably in the hospital for maybe few extra days compared to the endoscopic approach. But in general, babies do very well from the surgery.
Bill Klaproth (host): So I'm sure for parents, this can be a scary time. What would you say to parents who have just received a diagnosis of craniosynostosis and are anxious about their child's health?
Dr. Paul Klimo: Sure. So I walk them through it and tell them exactly what's going on. Usually, I'm very upfront that the child needs corrective surgery. But in general, it's a very straightforward, very safe operation. The surgery is typically done in conjunction with my plastic surgery partner. It's always a two-part operation in the sense that I, as the neurosurgeon, am usually the one making the cuts in the bone and taking the bone off; plastic surgery is the one that typically reshapes and puts the bones back together. It's very well tolerated in kids. And it has an excellent success rate. Children tolerate it very, very well. So that usually takes care of a lot of the anxiety.
Bill Klaproth (host): Absolutely. Well, Dr. Klimo, thank you so much for your time. This is really interesting and fascinating, and you've been very informative. So again, thank you for your time. We appreciate it.
Dr. Paul Klimo: You're very welcome.
Bill Klaproth (host): And once again, this is Dr. Paul Kilmo. And to learn more about neurosurgery at Le Bonheur, please visit lebonheur.org/neuroscience. And be sure to subscribe to the Peds Pod on Apple Podcasts, Google Podcasts, or wherever you listen to your podcasts. You can also check out lebonheur.org/podcast to view our full podcast library. And if you found this podcast helpful, please share it on your social channels. This is the Peds Pod by Le Bonheur Children's Hospital. Thanks for listening.