Craniosynostosis


 

What is it?

A normal skull is made up of multiple bones connected with seams or “sutures”. This allows a baby’s head to be flexible, making it easier for them to come out of the birth canal, as well as allow the brain to grow at a rapid rate. These sutures also serve as growth centers which make new bone. Normally, the sutures do not close or “fuse” until you are an adult. The metopic suture (in the middle of the forehead), is the only exception, and usually closes by 9 months of age. “Craniosynostosis” occurs if any of these sutures fuse before they are supposed to. This is important, because the skull no longer grows along the fused suture. As a result, the skull grows too much along the rest of the sutures in compensation to keep up with the growth of your child’s brain. Unlike positional plagiocephaly, which will usually get better as your child gets older, craniosynostosis tends to get worse, because the skull continues to grow in its abnormal pattern.

Thus, it is important to diagnosis it early. The earlier we are able to intervene, the better the results, because further abnormal growth is prevented. The specific suture that is fused usually produces a fairly characteristic head-shape, each of which has its own unique treatment. Most commonly, one suture is fused. However, there are specific syndromes that occur where multiple sutures can be fused. This is called “syndromic craniosynostosis”.

 

 

 

If the metopic suture is fused, your child will have “trigonocephaly”. This means that the forehead is pointed (like the keel of a boat), the eyes can appear closer together, and the head can appear pinched or narrow at the temples.

If the sagittal suture is closed, your child will have “scaphocephaly”. This is the most common type of single suture craniosynostosis. Scaphocephaly means that the head will appear very long, while also very narrow from side to side. Often the forehead will appear very prominent, and there may be a bulge at the back of the head. 

There are two coronal sutures. One (unicoronal) or both (bicoronal) may be fused. Unless your child has a syndrome, it is much more common to have unicoronal craniosynostosis. If this is the case, your child will typically have a flat forehead on one side and that eye and eyebrow will appear higher or raised. Sometimes the muscles in that eye will also be weak, which makes your child appear cross-eyed and causes them to tilt their head. The nose will also appear crooked.

 

There are also two lambdoid sutures. This is the least common type of craniosynostosis, making up only 1-3% of all cases. Typically, only one of the sutures is fused. This causes flattening of the back of the head, an abnormal position of one of the ears with bulging of the mastoid bone, and prominence of the forehead on the opposite side. When we see your child in the office, we will perform a detailed physical exam looking for these special findings and obtain a CT scan of your child’s head to help confirm a specific diagnosis.

Why do we treat craniosynostosis?

There are two general reasons to treat craniosynostosis. First, is the appearance of your child. Craniosynostosis can lead to head shapes which are quite abnormal and deforming, and this tends to only get worse as your child continues to grow. While your child could likely lead a normal life without any intervention, it is more realistic to think that their social and personal development will be negatively affected, especially by the time they reach their teens. It is much easier to treat the problem when your child is young. Not only are the results better, but it may prevent them from experiencing teasing or negative social interactions. Recovery also tends to be easier at a younger age because they tend not to remember their surgical experiences. The second reason to treat craniosynostosis is the potential for developing increased pressure around the brain (“ICP”). It is difficult to know how frequently this phenomenon occurs since we do not have very accurate ways of measuring increased ICP in these children. We think it happens in about 15% of children with single suture craniosynostosis, and over 40% of children with more than one fused suture. We do not have a way of predicting or knowing in which children it will develop, and sometimes it can happen very quickly. If it does develop, it can affect your child’s growth and development and, if left untreated, could ultimately lead to blindness or death.

What are the available treatment options?

Treatment varies depending on the type of craniosynostosis that your child has. Once a diagnosis is confirmed, we will have a lengthy conversation with you in the office about your options. We will then refer you to a neurosurgeon for evaluation, as well as an ophthalmologist. When there is increased pressure around the brain, it can cause swelling of the optic nerve called “papilledema”. This is something that the ophthalmologist can check for by looking in the back of your child’s eyes. The neurosurgeon will also check for any other concerns or signs of increased pressure related to the craniosynostosis, and will also evaluate you for surgery. Since surgery involves operating on the skull and around the brain, we perform all procedures along with a neurosurgeon.
If your child has any symptoms or signs of increased ICP, surgery will be necessary. If your child is developing normally and does not appear to have signs of increased ICP, you may choose whether to proceed with surgery or observation with very close and regular follow-up. However, as mentioned above, the earlier that surgery is performed, the better the outcome, the easier the surgery, the easier the recovery, and the least psychosocial effects on your child. Additionally, once your child is older than 4 months, we no longer have the option of performing a minimally invasive surgery.

What happens during surgery/recovery? 

 

  • Metopic Craniosynostosis


As mentioned above, with metopic craniosynostosis, most of the abnormality is found in the forehead and the temples, so these are the areas that we focus on during surgery. It is also important to realize that metopic craniosynostosis presents with a spectrum of severity. If your child’s deformity is mild, surgery may not be recommended. There are two general surgical options depending on your child’s age. The first is a minimally invasive strip craniectomy and the second is a fronto-orbital advancement (“FOA”), which is a form of open cranial vault remodeling.

A strip craniectomy takes advantage of the moldable nature of a baby’s skull within the first several months of life, and requires commitment to using a post-operative helmet. If your child is older than 4-5 months, they have missed the window for this procedure to be effective. Ideally, we perform this surgery at 3 months of age. Surgery involves making one small incision hidden in the hairline. Through this, we are able to lift the scalp off of the skull from the soft spot down to the root of the nose. The neurosurgeon then carefully separates the brain from the skull, and together we remove the abnormal suture and skull bone. This will leave a noticeable gap or dent in your baby’s forehead which will gradually fill in with bone over the next year. The incision is then closed with absorbable sutures and covered with skin glue. In general, the surgery takes 1-2 hours. Babies skulls are very vascular, so we expect some bleeding during the procedure. You should be prepared that your child may need a blood transfusion, either during surgery, or within the first 48 hours after surgery. If you are interested, directed blood donation is an option before the surgery, and we can discuss this when you come to the office.


Your child will then spend one night in the ICU for close monitoring, and will have a catheter in the bladder, and a special IV in the arm (arterial line). You will be able to feed and hold your child normally, but should expect that they may be irritable and painful. This usually improves significantly within the first 1-2 days. Within the first week, your child will be essentially back to normal. Be prepared that your child’s face and eyes will swell. This often becomes progressively worse over the first 48-72 hours, and the eyes may swell shut. Your child will often be more irritable as a result. If everything looks good the day after surgery, the catheter and arterial line will be removed. You will be able to go home once your child is eating well, pain is controlled, and the swelling has decreased enough for them to open their eyes. After the strip craniectomy, patients are often able to go home after only 1-2 days in the hospital. Within the week after surgery, you will need to see the orthotist, and they will begin fitting your child for a helmet. Once the helmet is ready, it will need to be worn 23 hours a day until your child is approximately one year old. It will take time for your child’s head shape to change as the helmet molds it over the next year.

 

An FOA is a larger, more involved surgery, which removes the abnormal bones, re-shapes them, and puts them back in a new position. Because of this, we have more control over the overall shape of the head, and no helmet is needed after surgery. In order to do the procedure properly, we want the bones to be a little stronger, so the surgery is usually not performed until 9-12 months of age. Surgery involves making a long wavy incision from ear to ear over the top of the head. Once healed, it is usually well hidden by the hair. The scalp is then lifted off of the skull down to the level of the eye sockets and the root of the nose. Together with the neurosurgeon, we then remove the top of the skull, the bones of the forehead, and the top of the eye sockets. The bones are then re-shaped and put back into place with a combination of sutures and absorbable plates and screws. The scalp is then closed with absorbable sutures and skin glue. The surgery generally takes 4-6 hours. Because this is a larger longer surgery than the strip craniectomy, a blood transfusion is usually necessary. Overall, the hospital experience will be similar to the strip craniectomy, except that there is generally more swelling, and you should plan for a 4-5 day stay, rather than 1-2.

After surgery, the swelling will gradually resolve over the next 1-2 weeks, at which time your child should generally be back to their baseline. Low grade fevers are very common in the first 2 weeks after surgery as well. You will be able to bathe your child normally, but we ask you to refrain from swimming or completely submerging their incisions in water till completely healed. If there is skin glue over the incisions, you will not need to perform any special wound care. Otherwise, we will ask you to apply ointment to the incisions twice a day. Overall, you should not worry about touching your child’s head, though it will feel different. They can play and return back to their normal activity, but you should protect them from any trauma to the head, sports, or rough housing. The stitches will dissolve over a few weeks. You should see an immediate change in the shape of your child’s head, but it will continue to grow and change over the next year. There may be prominent areas, lumps, bumps and soft spots that you feel. These are normal, and should gradually smooth and fill in as the skull heals. We will see you in the office more frequently within the first year after surgery, and then yearly for the first 5 years, as long as everything is going well. Once your child is healed, they will have no restrictions, and will be able to play sports without any issues.

 

  • Sagittal Craniosynostosis


As mentioned above, with sagittal craniosynostosis the head is narrow and long, so these are the areas that we focus on during surgery. There are two general surgical options depending on your child’s age. The first is a “minimally invasive strip craniectomy” and the second is a “cranial vault remodeling”. 

A strip craniectomy takes advantage of the moldable nature of a baby’s skull within the first several months of life, and requires commitment to using a post-operative helmet. If your child is older than 4-5 months, they have missed the window for this procedure to be effective. Ideally, we perform this surgery at 3 months of age. Surgery involves making two small incisions hidden in the hairline. Through this, we are able to lift the scalp off of the skull. The neurosurgeon then carefully separates the brain from the skull, and together we remove a wide strip of bone from the center of the skull, as well as two wedges down each side of the skull for a total of four. This allows the remaining skull bones to completely expand and bend and take full advantage of helmet remolding. You will immediately notice a change in the shape of your child’s head which will continue to change over the next year. You will also notice soft spots where the bone has been removed which will gradually fill in with bone over the next year. The incisions are then closed with absorbable sutures and covered with skin glue. In general, the surgery takes 1-2 hours. Babies skulls are very vascular, so we expect some bleeding during the procedure. You should be prepared that your child may need a blood transfusion, either during surgery, or within the first 48 hours after surgery. If you are interested, directed blood donation is an option before the surgery, and we can discuss this when you come to the office.

Your child will then spend one night in the ICU for close monitoring, will have a catheter in the bladder, and a special IV in the arm (arterial line). You will be able to feed and hold your child normally, but should expect that they may be irritable and painful. This usually improves significantly within the first 1-2 days. Within the first week, your child will be essentially back to normal. Be prepared that your child’s face and eyes will swell. This often becomes progressively worse over the first 48-72 hours, and the eyes may swell shut. Your child will often be more irritable as a result. If everything looks good the day after surgery, the catheter and arterial line will be removed. You will be able to go home once your child is eating well, pain is controlled, and the swelling has decreased enough for them to open their eyes. After the strip craniectomy, patients are often able to go home after only 1-2 days in the hospital. Within the week after surgery, you will need to see the orthotist, and they will begin fitting your child for a helmet. Once the helmet is ready, it will need to be worn 23 hours a day until your child is approximately one year old. It will take time for your child’s head shape to change as the helmet molds it over the next year.

A cranial vault remodeling is a larger, more involved surgery where the abnormal bones are removed, re-shaped, and put back in a new position. No helmet is needed after surgery. In order to do this properly, we want the bones to be a little stronger, so the surgery is usually performed at 9-12 months of age. If your child is less than 2 years old, research has shown that re-shaping the back of the head allows the front to usually remodel on its own. If your child is older than 2, or the deformity is very severe, we usually need to re-shape the entire skull. Surgery involves making a long wavy incision from ear to ear over the top of the head. Once healed, it is usually well hidden by the hair. The scalp is then lifted off of the skull. Together with the neurosurgeon, we then remove the necessary portions of the skull. The bones are then re-shaped and put back into place with a combination of sutures and absorbable plates and screws. The scalp is then closed with absorbable sutures and skin glue. The surgery generally takes 4-6 hours. Because this is a larger, longer surgery than the strip craniectomy, a blood transfusion is usually necessary. Overall, the hospital experience will be similar to the strip craniectomy, except that there is generally more swelling, and you should plan for a 4-5 day stay, rather than 1-2.

After surgery, the swelling will gradually resolve over the next 1-2 weeks, at which time your child should be back to their baseline. Low-grade fevers are very common in the first 2 weeks after surgery as well. You will be able to bathe them normally, but we ask you to refrain from swimming or completely submerging their incisions in water till completely healed. If there is skin glue over the incisions, you will not need to perform any special wound care. Otherwise, we will ask you to apply ointment to the incisions twice a day. Overall, you should not worry about touching your child’s head, though it will feel different. They can play and return back to their normal activity, but you should protect them from any trauma to the head, sports, or rough housing. The stitches will dissolve over a few weeks. You should see an immediate change in the shape of your child’s head, but it will continue to grow and change over the next year. There may be prominent areas, lumps, bumps and soft spots that you feel. These are normal and should gradually smooth and fill in as the skull heals. We will see you in the office more frequently within the first year after surgery, and then yearly for the first 5 years, as long as everything is going well. Once your child is healed, they will have no restrictions and will be able to play sports without any issues.

 

  • Coronal Craniosynostosis

 

With coronal craniosynostosis, the main abnormal areas are the forehead, brow, and eyes, so this is what we focus on correcting during surgery. Some centers will consider performing a strip craniectomy with post-operative helmet therapy. However, it is a general consensus that adequately correcting the orbital deformity is very difficult without performing an open cranial vault procedure. Therefore, we perform a “fronto-orbital advancement” or “FOA” for these patients. In order to do this properly, we want the bones to be a little stronger, so the surgery is usually performed at 9-12 months of age. Surgery involves making a long wavy incision from ear to ear over the top of the head. Once healed, it is usually well hidden by the hair. The scalp is then lifted off of the skull down to the level of the eye sockets and the root of the nose. Together with the neurosurgeon, we then remove the top of the skull, the bones of the forehead and the top of the eye sockets. The bones are then re-shaped and put back into place with a combination of sutures and absorbable plates and screws. Because the skull continues to grow and re-shape as your child gets older, we initially overcorrect the position of the abnormal eye socket/forehead. Because of this, the brow may initially look very prominent, but your child will grow into it with time. The scalp is then closed with absorbable sutures and skin glue. The surgery generally takes 4-6 hours. Babies skulls are very vascular, so we expect some bleeding during the procedure. You should be prepared that your child will likely need a blood transfusion, either during surgery, or within the first 48 hours after surgery. If you are interested, directed blood donation is an option before the surgery, and we can discuss this when you come to the office.

Your child will then spend one night in the ICU for close monitoring, and will have a catheter in the bladder, and a special IV in the arm (arterial line). You will be able to feed and hold your child normally, but should expect that they may be irritable and painful. This usually improves significantly within the first 1-2 days. Within the first week, your child will be essentially back to normal. Be prepared that your child’s face and eyes will swell. This often becomes progressively worse over the first 48-72 hours, and the eyes may swell shut. Your child will often be more irritable as a result. If everything looks good the day after surgery, the catheter and arterial line will be removed and you will be transferred to a regular room. You will be able to go home once your child is eating well, pain is controlled, and the swelling has decreased enough for them to open their eyes. Expect that you will be in the hospital for 4-5 days. If your child’s eye muscles are abnormal in the affected eye, this will not be improved with surgery and may get worse. Treatment will be provided by your ophthalmologist and may require additional eye surgery in the future.

After surgery, the swelling will gradually resolve over the next 1-2 weeks, at which time your child should be back to their baseline. Low-grade fevers are very common in the first 2 weeks after surgery as well. You will be able to bathe them normally, but we ask you to refrain from swimming or completely submerging their incisions in water till completely healed. If there is skin glue over the incisions, you will not need to perform any special wound care. Otherwise, we will ask you to apply ointment to the incisions twice a day. Overall, you should not worry about touching your child’s head, though it will feel different. They can play and return back to their normal activity, but you should protect them from any trauma to the head, sports or rough housing. The stitches will dissolve over a few weeks. You should see an immediate change in the shape of your child’s head, but it will continue to grow and change over the next year. There may be prominent areas, lumps, bumps and soft spots that you feel. These are normal and should gradually smooth and fill in as the skull heals. We will see you in the office more frequently within the first year after surgery, and then yearly for the first 5 years, as long as everything is going well. Once your child is healed, they will have no restrictions and will be able to play sports without any issues.

 

  • Lambdoid Craniosynostosis


Lambdoid craniosynostosis is rare and somewhat difficult to treat. Most of the head shape abnormality is in the back of the head, and behind one of the ears. However, the longer the process goes on, the more the entire head and face become abnormal as well. Therefore, surgery aims to halt this abnormal process to minimize future deformity, and attempts to make the back of the head more symmetric. The bulging behind the ear is very difficult to fix because of its location and is usually not changed much by the surgery. Once there is asymmetry of the face, it is very difficult to treat or change as well. Often, lambdoid craniosynostosis is not identified till a child is older. This is because the diagnosis is so rare, and often mimics positional plagiocephaly early on till the abnormality becomes more severe. If the child does present younger than 5 months of age, a strip craniectomy with helmet therapy might be considered (please see the sagittal craniosynostosis section for details). However, the surgery generally offered is called a “posterior cranial vault remodeling”.

In order for the procedure to be done properly, we want the bones to be a little stronger, so the surgery is usually performed at 9-12 months of age. Surgery involves making a long wavy incision from ear to ear over the top of the head. Once healed, it is usually well hidden by the hair. The scalp is then lifted off of the back of the skull. Together with the neurosurgeon, we then remove the abnormal bones of the back of the skull. The bones are then re-shaped, and the flat side of the skull is made wider. The bones are put back into place with a combination of sutures and absorbable plates and screws. The scalp is then closed with absorbable sutures and skin glue. The surgery generally takes 4-6 hours. Babies skulls are very vascular, so we expect some bleeding during the procedure. You should be prepared that your child will likely need a blood transfusion, either during surgery or within the first 48 hours after surgery. If you are interested, directed blood donation is an option before the surgery, and we can discuss this when you come to the office.

Your child will then spend one night in the ICU for close monitoring, and will have a catheter in the bladder, and a special IV in the arm (arterial line). You will be able to feed and hold your child normally, but should expect that they may be irritable and painful. This usually improves significantly within the first 1-2 days. Within the first week, your child will be essentially back to normal. Be prepared that your child’s face and eyes will swell, but usually less than when we operate on the front of the head. This often becomes progressively worse over the first 48-72, and the eyes may swell shut. Your child will often be more irritable as a result. If everything looks good the day after surgery, the catheter and arterial line will be removed and you will be transferred to a regular room. You will be able to go home once your child is eating well, pain is controlled, and the swelling has decreased enough for them to open their eyes. Expect that you will be in the hospital for 4-5 days.

After surgery, the swelling will gradually resolve over the next 1-2 weeks, at which time your child should be back to their baseline. Low-grade fevers are very common in the first 2 weeks after surgery as well. You will be able to bathe them normally, but we ask you to refrain from swimming or completely submerging their incisions in water till completely healed. If there is skin glue over the incisions, you will not need to perform any special wound care. Otherwise, we will ask you to apply ointment to the incisions twice a day. Overall, you should not worry about touching your child’s head, though it will feel different. They can play and return back to their normal activity, but you should protect them from any trauma to the head, sports or rough housing. The stitches will dissolve over a few weeks. You should see an immediate change in the shape of your child’s head, but it will continue to grow and change over the next year. There may be prominent areas, lumps, bumps and soft spots that you feel. These are normal and should gradually smooth and fill in as the skull heals. We will see you in the office more frequently within the first year after surgery, and then yearly for the first 5 years, as long as everything is going well. Once your child is healed, they will have no restrictions and will be able to play sports without any issues.

Strip Craniectomy versus Cranial Vault Remodeling - Which one should we choose?

There are pros and cons to both procedures, and these differ depending on whether your child has metopic or sagittal craniosynostosis.  As parents, you must weigh them carefully in order to make the decision that is right for you and your family.  In general, the strip craniectomy is a shorter, less invasive procedure, with less blood loss than the cranial vault remodeling.  This leads to a shorter hospital stay, and less chance of a blood transfusion.  It also results in smaller incisions and scars.  However, it can only be done if your child is in the ideal window for surgery (<5 months of age, and ideally 3 months of age) and you must be willing to commit to approximately a year of postoperative helmet therapy.  This means frequent trips to the orthotist for helmet adjustments, and the helmet needs to be kept clean.  Some parents are also uncomfortable with the stigma that can be associated with a child wearing a helmet.  However, most families get through this time period extremely successfully and find it very worthwhile in the end.  Cranial vault remodeling, on the other hand, is a larger, longer procedure, but you do not have to deal with the burden of helmet therapy.  The results of the two procedures are also not entirely uniform.  For sagittal craniosynostosis, the strip craniectomy has the potential to create a totally normal head shape, and often has better results than the open procedure.  This is largely because the early intervention addresses and prevents the deformity of the forehead and temples which is difficult to correct later on.  The open cranial vault remodeling has the potential for excellent results as well, but may not completely normalize the appearance of the forehead and temples.  For metopic craniosynostosis, on the other hand, the strip craniectomy has the potential for excellent results, but is often not as predictable as the open surgery. This is because it is depends on brain growth to help remodel the bone, which occurs less in the front of the skull.  If the results are not as dramatic as expected, the open surgery may still be necessary later on.

What are the risks?

As mentioned above, you should be prepared for the likelihood that your child will need a blood transfusion during or after surgery. Directed blood donation is possible if you are interested. Surgery for craniosynostosis is done fairly frequently, and the vast majority of children do extremely well. However, these procedures should not be taken lightly as they do involve operating on the skull and around the brain. Though rare, serious complications such as severe bleeding, brain injury, leaking of cerebrospinal fluid, seizure, air embolism, stroke, or even death could occur. We take every precaution to prevent these complications and work closely with a neurosurgeon, operative staff, and nursing staff which are all familiar with these procedures.

After any surgery, there is always a potential risk for infection, which could affect the way the bones heal. This is also uncommon but is the reason we give your child antibiotics for the first few days after surgery, and send you home with antibiotics if your child has had one of the larger open procedures.

Though children usually heal quite well after surgery, it is possible that there may still be some contour irregularities, asymmetries, or small holes left that don’t fill in with bone. These are called “calvarial defects”. Many of these spots will smooth or fill in with time, so we tend to perform watchful waiting before considering a revision surgery. If your child is approximately 4 years old, and still has areas that have yet to heal or could be improved, then we might consider another more minor revision surgery before school age. More significant residual deformities can occasionally occur which might necessitate a larger revision surgery, or sometimes can be camouflaged later in life with custom implants or fat grafting.

It is also possible that the scalp scar may become stretched or wide as it heals. The scar will not grow hair, so it may be obvious in children with short haircuts. If this becomes a problem, the scar can easily be revised in a short outpatient procedure.
Occasionally, as the plates and screws are absorbing, they may cause lumps, bumps, soft spots, or irritation. This tends to occur 12-14 months after surgery and usually resolves on its own without intervention.


How does helmet therapy work? 

A helmet may be prescribed to your child for severe positional plagiocephaly, or post-operatively after a strip craniectomy. The helmet works by harnessing the moldable nature of your child’s skull and rapid brain growth in the first year of life. The helmet is uniquely designed for your child’s head shape. It fits snugly in areas that we don’t want to grow and leaves space in the areas that we want to change. By doing so, we are able to gradually shape the head in an ideal way. Since your child is growing quickly, this means that the helmet will have to be adjusted frequently. This may mean re-shaping the inner padding of the helmet, or fabricating a new helmet. Most children will go through several helmets during the length of their therapy. The helmet is generally worn till your child is approximately one year of age. Research has shown that there does not seem to be much additional benefit gained in head shape by wearing the helmet past a year. In order to get the maximum benefit from the helmet therapy, it should be worn 23 hours a day, with breaks only for bath time and cleaning. Children generally adjust very quickly to the helmet, and it usually does not bother them. The helmet itself is fabricated by a specialist called an orthotist or prosthetist. There are several companies which perform this service. We work with a company called Star Cranial. Once the helmet is prescribed, you will meet with your orthotist, and they will take measurements and images of your child’s head. They will work with us to come up with a specialized plan to create the desired head shape. All adjustments to the helmet and new helmets will be provided by your orthotist, so you will need to see them frequently. Helmets can be customized in a variety of colors and designs.

 

Multi-suture/Syndromic Craniosynostosis

Most cases of craniosynostosis that we see are “isolated” or “non-syndromic”, meaning that only one suture is fused, and the child is otherwise generally healthy. More rarely, craniosynostosis can be seen as part of a specific syndrome. “Syndromic Craniosynostosis” occurs in 1/25, 000 to 1/150,000 births. These children tend to have numerous medical problems which vary depending on the syndrome. Usually more than one suture is fused. The growth of the facial bones is also abnormal which makes the problem much more difficult to treat. These children usually need multiple surgeries by the time they reach adulthood. Syndromes include: Apert’s Syndrome, Crouzon’s Syndrome, Pfeiffer’s Syndrome, Muenke Syndrome, and Saethre-Chotzen Syndrome. Many of these children have either bicoronal craniosynostosis or multi-suture craniosynostosis which results in a skull that significantly restricts brain growth with a high risk for increased pressure around the brain. The main initial treatment goal is creating as much space as possible in the skull for the brain. This is typically accomplished with a procedure called “posterior cranial vault distraction” with excellent results (see separate section). This process can be repeated as necessary. Later in life, these children will generally need operations to reposition the forehead and eye-sockets, as well as the cheek bones, nose, and jaws. They will also typically need other neurosurgical procedures as well as operations on other parts of the body like the heart, and the feet and hands. If your child is diagnosed with one of these syndromes, we will meet with you and discuss all the treatment options in detail, and work closely with the other specialists to come up with a custom plan that is right for you.

 

Posterior Cranial Vault Distraction Osteogenesis (PVDO)


PVDO is a technique used to gradually move the bones of the back of the skull to increase volume when the skull is so tight that there is not enough room for the brain and/or there is increased pressure around the brain. This generally occurs in patients with syndromic craniosynostosis where there is more than one suture that is fused in the skull. These children need significantly more room for their brains than traditional surgical techniques can provide. Moving the bones into the new position necessary would leave a large gap that would likely not heal, and the scalp would be too tight to close. Therefore, in these situations, we are able to use a process called “distraction”. First, cuts are made in the bones that we want to move which separates them from the rest of the skull. We then attach metal plates and screws to the bones which are connected to a metal tube that sticks out of the skin. Using a screwdriver, the metal tube is then turned, which slowly separates the bones over several weeks. Because the process is slow, the scalp gradually stretches, and new bone forms in the gap created. This lets us move the bones much farther, and the end result is more stable. Usually the distractor is “turned” twice a day for a total of 2 mm. The distraction process is fairly easy, and most of the distraction can be done at home by the parents once a comfort level has been achieved. We will have you keep a log of how much you turn the device each day. We will periodically get x-rays or CT scans throughout the treatment period to see how the hardware and bones are moving, and decide when we are ready to stop distraction. Once distraction is finished, the devices are kept in place for 6 weeks to 2 months to allow the bone to become solid. Once the bone is healed, we bring your child back to the operating room for a short procedure to remove the metal devices. This is a process that can be repeated again if necessary. When these children are older, they will need further surgeries to address the shape of the face and front of the skull.

 

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