About 1 in 2,000 babies is affected by craniosynostosis, in which the sutures of the skull bones close too early. This leads to an abnormally shaped head not related to external factors.
Craniosynostosis can be hereditary and accompany other congenital defects or occur randomly in otherwise healthy children with normal intelligence.
Listen as Kamlesh Patel, MD, Washington University pediatric plastic and reconstructive surgeon at St. Louis Children’s Hospital, explains how this condition is treated and how the patient might be affected in the long term.
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Pediatric Craniosynostosis: Abnormal Head Shape in Infants
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Learn more about Kamlesh Patel, MD
Kamlesh Patel, MD
Kamlesh Patel, MD specializes in craniofacial and pediatric plastic surgery. His practice includes the treatment of both adults and children for cleft lip and palate disorders, reconstruction for craniosynostosis and other facial/skull malformations, treatment of moles and vascular malformations, ear reconstruction for children with abnormal or absent ears, and facial trauma reconstruction.Learn more about Kamlesh Patel, MD
Transcription:
Pediatric Craniosynostosis: Abnormal Head Shape in Infants
Melanie Cole (Host): About 1 in 2,000 babies is affected by craniosynostosis in which the sutures of the skull bone close too early. This leads to an abnormally shaped head not related to external factors. My guest today is Dr. Kamlesh Patel. He’s a Washington University pediatric, plastic and constructive surgeon at St. Louis Children’s Hospital. Welcome to the show, Dr. Patel. What is craniosynostosis?
Dr. Kamlesh Patel (Guest): Craniosynostosis, just going back—first, I’ll explain the anatomy of the skull. The best way to think of the skull is it’s just not one piece of bone. It’s basically multiple pieces of bone that are connected by fibrous tissue called “sutures”. The reason why you don’t want one piece of bone is that brain is doubling in size in the first six months. So, to kind of meet that rapid brain growth, you want the bones to be separate and just connected loosely. Craniosynostosis happens when one of these sutures closes too early. This always happens before birth, in utero. It’s never something that happens after birth. How often does it happen? As you mentioned, it’s 1 in 2,000.In terms of craniosynostosis, I don’t know if you want to go into the diagnosis already, but pretty much the diagnosis can be made clinically, either by a neurosurgeon or a craniofacial surgeon. Usually, when you think of the sutures, how the head shape is when the suture fusion is--I’d say the most common one is on top of the head just straight down the middle on the top of the head called the “sagittal suture”. When that suture closes early, it prevents growth perpendicular to the suture. So, what do you end up? You end up with a head that’s narrow and then because the other sutures are open, the brain’s always going to find somewhere else to grow. It grows more front and back, so you end up with a very long head. That’s just an example of the most common one. That’s why, with each particular suture that closes, we kind of know the head shape that’s expected with each one.
Melanie: Is this something that can be diagnosed during pregnancy in utero?
Dr. Patel: It’s difficult. In most cases, actually, in pretty much all cases, it’s not diagnosed in utero. It’s something that's diagnosed pretty much at birth or pretty soon after birth.
Melanie: And, can it affect the brain growth itself?
Dr. Patel: That’s one of the debates. Definitely that’s the question is, why does this need to be fixed? You definitely have the cosmetic concerns with the changes it’s having on the head shape, in particular some of the more frontal sutures, the impact it has on the face. So, definitely, that’s one reason why you’d fix this. The second reason is, what impact can it have on the brain? There’s definitely a rate of around 10-30% chance of having increased pressure meaning not enough room for the brain to completely grow in the skull because it’s inhibited by the fused suture. The way I usually like to think about it, if it’s usually one suture, the brain usually finds somewhere else to grow. That’s where you get the abnormal head shape. I tend to worry more about the ones that have more than one suture fused—kind of a double head. Now, you’re struggling to find the room for the brain. Those are the ones we tend to worry about more when it ones to increased pressure.
Melanie: Who would notice this first? Would it be the obstetrician in the delivery room? And then, when would a specialist such as yourself come into play?
Dr. Patel: Usually, I’d say the diagnosis, sometimes for the obstetrician Is difficult, especially straightaway after birth. At times, the head shape is a little bit abnormal and then it fixes itself pretty quickly after that. I think most cases, the parents are very smart. They kind of pick up that the head shape is abnormal and then they go to their pediatrician, and the pediatrician kind of picks up, then, after that that the head shape is abnormal and a referral is made to either craniofacial surgeon or a pediatric neurosurgeon that specializes in this.
Melanie: Tell us about the management of the condition.
Dr. Patel: In terms of management, as soon as we see the patient, in Louis Children’s, it’s a team approach. The reason why we do a team approach between myself, the craniofacial surgeon and the neurosurgeon is that the outcomes are better when we work together, the safety is improved and also the head shape outcome is better when we just work together. In terms of management, we’ve done quite a lot of education to our local pediatricians and also nationally to get the referral as soon as possible, and I’ll explain why that’s important. Traditionally, craniosynostosis you think, let’s see it fixes itself, wait until at least five to six months, and then make the referral. Our preference is to get the referral early, and the reason why is because we actually offer two different treatments for this population. One is what we call, minimally invasive--the endoscopic route. If you remember, I mentioned that the brain doubles in size in the first six months. The way the endoscopic route works is, you just take out the fused suture, and then, with the use of a helmet post-operatively, you guide the brain growth. You guide the skull growth itself. So then, it kind of fixes itself but you need that rapid brain growth rate which is up to about is up to about six months to really take advantage of that. So, if someone comes to us after about five to six months of age, we say that outside the minimally invasive approach and we go to the more traditional approach which is tried and tested and the most common technique everyone else uses in the country. With the open approach, what we do is make an incision from ear to ear. The reason why we make it in the hairline is because it hides the scar better. Then, you actually, with the aid of the neurosurgeon, we take the bones that are affected off or we contour them, and that’s where my job comes in, to put the bones back where they should be and create more room for the brain using dissolvable plates--plates that dissolve on their own over a year to hold everything in place. Usually, in terms of the differences between the two operations, definitely the endoscopic one is shorter. It’s usually a one-day stay in the hospital and less blood loss. The downside is you have to wear a helmet until one year of age. With the open operation, there’s definitely more work up front. It’s usually about two to three days in the hospital. Operations are a bit longer, but no helmet afterwards. We’ve done a lot of research looking at both. The end outcome is the same between the two operations. So, definitely, there’s nothing wrong with picking either option but, if you’re younger, we can give both options. If you come after five to six months of age, then we give younger option--I mean, then we go with the open operation.
Melanie: What would you like to tell other pediatricians about getting to that specialist early enough that you can use the endoscopic procedure and getting them in so that they see someone like you.
Dr. Patel: Yes. So, we always make it a priority when anyone makes a referral of craniosynostosis, the flag goes off, pretty much the referral’s made, and get them in as soon as possible. I advise if you get comfortable looking at these head shapes, if it’s one that usually doesn’t fit the patterns of, say, what we call deformational, you know, the one from sleeping on the back and causing the flattening along the back of the head, if you’re seeing something beyond that, the best thing is just make the referral early so the evaluation can be done. At our institution, we always like to get a CT scan just to confirm the diagnosis. The last thing you want to do is take a child to the operating room without confirming the diagnosis. Now, the CT scans are definitely quicker, the radiation protocols are lot lower, to the point where the radiation dose is just the same as living in St. Louis for six months. So, if you’re comfortable offering the CT scan just to make sure that we have the right diagnosis before going to the operating room.
Melanie: Tell us, in the last few minutes, about some of the lifelong considerations for a child with craniosynostosis.
Dr. Patel: So, these patients are followed in a craniofacial team until skeletal maturity. So, we always make sure that after the operation is done, whichever one it is, that the head’s growing okay, and that there’s no issues in terms of psychological evaluation. All of these children also get psychological and speech evaluation. Long-terms studies have shown that these children are pretty much within the normal range but we want to make sure that we follow these children and get them the resources they need. But, all in all, a lot of them actually do just fine as they grow.
Melanie: Tell us about your team. Why is St. Louis Hospital so great to work with?
Dr. Patel: So, here, we’re one of the oldest and one of the largest teams in the Midwest. We have a long history of safety with this procedure. Even though the operation is a big operation, it can be done safely. We published our outcomes just last year over the past 10 years. I think that’s the key. You just want to make sure that the center has a high-volume of this, and that it can be done safely.
Melanie: Thank you so much for being with us today. A physician can refer a patient by calling Children’s direct physician access line at 1-800-678-HELP. That’s 1-800-678-4357. You’re listening to Radio Rounds with St. Louis Children’s Hospital. For more information on resources available at St. Louis Children’s Hospital, you can go to www.stlouischildrens.org. That’s www.stlouischildrens.org. This is Melanie Cole. Thanks so much for listening.
Pediatric Craniosynostosis: Abnormal Head Shape in Infants
Melanie Cole (Host): About 1 in 2,000 babies is affected by craniosynostosis in which the sutures of the skull bone close too early. This leads to an abnormally shaped head not related to external factors. My guest today is Dr. Kamlesh Patel. He’s a Washington University pediatric, plastic and constructive surgeon at St. Louis Children’s Hospital. Welcome to the show, Dr. Patel. What is craniosynostosis?
Dr. Kamlesh Patel (Guest): Craniosynostosis, just going back—first, I’ll explain the anatomy of the skull. The best way to think of the skull is it’s just not one piece of bone. It’s basically multiple pieces of bone that are connected by fibrous tissue called “sutures”. The reason why you don’t want one piece of bone is that brain is doubling in size in the first six months. So, to kind of meet that rapid brain growth, you want the bones to be separate and just connected loosely. Craniosynostosis happens when one of these sutures closes too early. This always happens before birth, in utero. It’s never something that happens after birth. How often does it happen? As you mentioned, it’s 1 in 2,000.In terms of craniosynostosis, I don’t know if you want to go into the diagnosis already, but pretty much the diagnosis can be made clinically, either by a neurosurgeon or a craniofacial surgeon. Usually, when you think of the sutures, how the head shape is when the suture fusion is--I’d say the most common one is on top of the head just straight down the middle on the top of the head called the “sagittal suture”. When that suture closes early, it prevents growth perpendicular to the suture. So, what do you end up? You end up with a head that’s narrow and then because the other sutures are open, the brain’s always going to find somewhere else to grow. It grows more front and back, so you end up with a very long head. That’s just an example of the most common one. That’s why, with each particular suture that closes, we kind of know the head shape that’s expected with each one.
Melanie: Is this something that can be diagnosed during pregnancy in utero?
Dr. Patel: It’s difficult. In most cases, actually, in pretty much all cases, it’s not diagnosed in utero. It’s something that's diagnosed pretty much at birth or pretty soon after birth.
Melanie: And, can it affect the brain growth itself?
Dr. Patel: That’s one of the debates. Definitely that’s the question is, why does this need to be fixed? You definitely have the cosmetic concerns with the changes it’s having on the head shape, in particular some of the more frontal sutures, the impact it has on the face. So, definitely, that’s one reason why you’d fix this. The second reason is, what impact can it have on the brain? There’s definitely a rate of around 10-30% chance of having increased pressure meaning not enough room for the brain to completely grow in the skull because it’s inhibited by the fused suture. The way I usually like to think about it, if it’s usually one suture, the brain usually finds somewhere else to grow. That’s where you get the abnormal head shape. I tend to worry more about the ones that have more than one suture fused—kind of a double head. Now, you’re struggling to find the room for the brain. Those are the ones we tend to worry about more when it ones to increased pressure.
Melanie: Who would notice this first? Would it be the obstetrician in the delivery room? And then, when would a specialist such as yourself come into play?
Dr. Patel: Usually, I’d say the diagnosis, sometimes for the obstetrician Is difficult, especially straightaway after birth. At times, the head shape is a little bit abnormal and then it fixes itself pretty quickly after that. I think most cases, the parents are very smart. They kind of pick up that the head shape is abnormal and then they go to their pediatrician, and the pediatrician kind of picks up, then, after that that the head shape is abnormal and a referral is made to either craniofacial surgeon or a pediatric neurosurgeon that specializes in this.
Melanie: Tell us about the management of the condition.
Dr. Patel: In terms of management, as soon as we see the patient, in Louis Children’s, it’s a team approach. The reason why we do a team approach between myself, the craniofacial surgeon and the neurosurgeon is that the outcomes are better when we work together, the safety is improved and also the head shape outcome is better when we just work together. In terms of management, we’ve done quite a lot of education to our local pediatricians and also nationally to get the referral as soon as possible, and I’ll explain why that’s important. Traditionally, craniosynostosis you think, let’s see it fixes itself, wait until at least five to six months, and then make the referral. Our preference is to get the referral early, and the reason why is because we actually offer two different treatments for this population. One is what we call, minimally invasive--the endoscopic route. If you remember, I mentioned that the brain doubles in size in the first six months. The way the endoscopic route works is, you just take out the fused suture, and then, with the use of a helmet post-operatively, you guide the brain growth. You guide the skull growth itself. So then, it kind of fixes itself but you need that rapid brain growth rate which is up to about is up to about six months to really take advantage of that. So, if someone comes to us after about five to six months of age, we say that outside the minimally invasive approach and we go to the more traditional approach which is tried and tested and the most common technique everyone else uses in the country. With the open approach, what we do is make an incision from ear to ear. The reason why we make it in the hairline is because it hides the scar better. Then, you actually, with the aid of the neurosurgeon, we take the bones that are affected off or we contour them, and that’s where my job comes in, to put the bones back where they should be and create more room for the brain using dissolvable plates--plates that dissolve on their own over a year to hold everything in place. Usually, in terms of the differences between the two operations, definitely the endoscopic one is shorter. It’s usually a one-day stay in the hospital and less blood loss. The downside is you have to wear a helmet until one year of age. With the open operation, there’s definitely more work up front. It’s usually about two to three days in the hospital. Operations are a bit longer, but no helmet afterwards. We’ve done a lot of research looking at both. The end outcome is the same between the two operations. So, definitely, there’s nothing wrong with picking either option but, if you’re younger, we can give both options. If you come after five to six months of age, then we give younger option--I mean, then we go with the open operation.
Melanie: What would you like to tell other pediatricians about getting to that specialist early enough that you can use the endoscopic procedure and getting them in so that they see someone like you.
Dr. Patel: Yes. So, we always make it a priority when anyone makes a referral of craniosynostosis, the flag goes off, pretty much the referral’s made, and get them in as soon as possible. I advise if you get comfortable looking at these head shapes, if it’s one that usually doesn’t fit the patterns of, say, what we call deformational, you know, the one from sleeping on the back and causing the flattening along the back of the head, if you’re seeing something beyond that, the best thing is just make the referral early so the evaluation can be done. At our institution, we always like to get a CT scan just to confirm the diagnosis. The last thing you want to do is take a child to the operating room without confirming the diagnosis. Now, the CT scans are definitely quicker, the radiation protocols are lot lower, to the point where the radiation dose is just the same as living in St. Louis for six months. So, if you’re comfortable offering the CT scan just to make sure that we have the right diagnosis before going to the operating room.
Melanie: Tell us, in the last few minutes, about some of the lifelong considerations for a child with craniosynostosis.
Dr. Patel: So, these patients are followed in a craniofacial team until skeletal maturity. So, we always make sure that after the operation is done, whichever one it is, that the head’s growing okay, and that there’s no issues in terms of psychological evaluation. All of these children also get psychological and speech evaluation. Long-terms studies have shown that these children are pretty much within the normal range but we want to make sure that we follow these children and get them the resources they need. But, all in all, a lot of them actually do just fine as they grow.
Melanie: Tell us about your team. Why is St. Louis Hospital so great to work with?
Dr. Patel: So, here, we’re one of the oldest and one of the largest teams in the Midwest. We have a long history of safety with this procedure. Even though the operation is a big operation, it can be done safely. We published our outcomes just last year over the past 10 years. I think that’s the key. You just want to make sure that the center has a high-volume of this, and that it can be done safely.
Melanie: Thank you so much for being with us today. A physician can refer a patient by calling Children’s direct physician access line at 1-800-678-HELP. That’s 1-800-678-4357. You’re listening to Radio Rounds with St. Louis Children’s Hospital. For more information on resources available at St. Louis Children’s Hospital, you can go to www.stlouischildrens.org. That’s www.stlouischildrens.org. This is Melanie Cole. Thanks so much for listening.