Many have asked:
"What will be done in Olivia's surgery?"
"What is wrong with Olivia?"
"Is surgery REALLY necessary?"
The answers to these questions and more are below. (copied from our doctors website)
It is a detailed read but very informative.

Single Suture Craniosynostoses
Overview:

There are four different kinds of single suture synostosis, with each type being caused by a different skull suture prematurely fusing shut. All the single sutural synostoses share some features in common, and these are discussed below. Following this, each type of synostosis (sagittal, coronal, metopic and lambdoid) is discussed individually. Finally, the surgical treatments for the single sutural craniosynostoses are reviewed.

To begin with, it is important to determine if a child truly has craniosynostosis because the most common cause for an abnormal skull shape is not craniosynostosis, but is positional plagiocephaly. Positional plagiocephaly (aka. a skull deformation) is discussed elsewhere on this site (Go to Deformations).

Skull Growth
In order to understand craniosynostosis, it is helpful to know a little bit about the normal growth of the skull. The skull is not made up from one single bone, but instead is made up of different bones. The junctions where these bones meet are called sutures. We know that sutures are very important in growing children. The skull does not usually grow on it’s own; instead, it only gets larger because the brain is growing and putting pressure on the skull to get bigger. So, if the brain does not grow, the skull does not get larger. In the first few years of life, the brain grows very quickly. As the brain grows, it stretches the skull bones apart, with the sutures acting like expansion joints. It is believed that this stretching of the sutures sends a signal to the skull bones telling them to grow bigger so that the skull can accommodate the enlarging brain (actually, the growth of the skull is slightly more complicated that this, and a simplified version is being presented here). If one of these sutures has fused shut, the skull cannot expand to accommodate the growing brain, so the brain must push the other non-fused sutures further apart so there is enough room for the brain. This sequence of events leads to the abnormal skull shape that either parents, or pediatricians first notice.

Fusion of the Suture

What causes sutures to fuse shut before they are supposed to? The two most common causes for premature closure of a suture are either a change in one of the child’s genes, or from outside pressure on the skull. When only one single suture is fused shut, the most common cause is probably “environmental” (or from outside pressure on the skull). We know that it is possible to cause craniosynostosis in animals by restricting skull growth while the animal is still in the womb, and it is very likely that this is the primary cause for the majority of the single sutural synostoses in babies. The uterus, or womb, is a relatively small space. We know that sometimes babies can end up in certain positions in the uterus where they cannot move around freely (for example, the breech position). We suspect that children born with a single sutural synostosis may have been positioned in the uterus so that there is pressure on a part of the skull. If the skull gets caught in a tight place in the uterus, this pressure can restrict the ability of the skull bones to be stretched apart by the growing brain. If the suture is not stretched apart, it “thinks” it’s job is done and it fuses shut with normal bone. Abnormal skull shapes and single sutural synostoses are more commonly seen in twins than in single births, further supporting this theory of in-utero constraint. Craniosynostosis is also more common in boys than girls leading some researchers to speculate that testosterone might make suture closure more likely if there is any restriction of skull growth inside the uterus. Mothers who have delivered babies with single sutural synostosis should not feel guilty that they did anything wrong during their pregnancy to cause this condition. Craniosynostosis occurs in spite of the mother doing everything “right.”

Usually, when children with a single sutural craniosynostosis grow up, they do not pass this trait on to their children. However, it has been reported (and we have seen cases of this, as well) of a single sutural synostosis being passed from generation to generation. This hereditary pattern suggests that the single sutural synostosis is caused by a problem with a gene allowing this trait to be passed from one family member to the next. It is possible to test for some of these genes to see if they are present in children with craniosynostosis (which is something that we are currently doing in Dallas). However, it should be remembered that the overwhelming majority of single sutural synostoses appear to be the product of two parents with normal genes, and a mother who has normal prenatal care. Should children born with a single sutural synostosis decide to have children of their own in adulthood, the chance of passing on the condition is estimated to be less than 2%. If a couple has a child who is born with a single sutural synostosis, the chance of this couple having a another child with a single sutural synostosis is also thought to be about 2%.

Problems Caused by Sutural Fusion
Does a single sutural synostosis affect a child’s development or intelligence? This is a difficult question to answer. At the current time, we believe it either does not affect a child’s mental development, or if it does, it cannot be measured by current testing. There are a number of studies that have been published, which examine the possibility that there may be raised pressure inside of the skull of children born with craniosynostosis. The concern is that if the growing brain is kept from enlarging inside the skull because a suture has closed prematurely, then as the space inside the skull gets more crowded, pressure can build up. Studies suggest that a small percentage of children born with a single fused suture will have raised intracranial (inside the skull) pressure. When a group of children with craniosynostosis were studied with I.Q. testing, it was noted that those children with the highest pressure had lower I.Q.s. The problem is that these were not well-designed studies. To begin with, no one is sure what the normal pressure is inside an infant’s skull, so it is hard to determine exactly what is a high pressure. In addition, we know that pressure inside the skull normally varies up and down and does not stay constant. Furthermore, in some of the studies showing that children with raised intracranial pressure have lower IQ’s, it turns out that those children with the lowest IQ’s had syndromes that are known to have an abnormal brain structure (in some syndromes, we know that the brain may not be wired normally). Finally, the I.Q. test examines only one form of intelligence. There are not good tests for creative intelligence, artistic intelligence, musical intelligence, social intelligence, “common sense”, and other types of intelligence.

There are also some studies that suggest that children with single sutural synostosis may have a higher rate of experiencing some minor developmental or behavioral problems. Once again, it is not clear that these studies are good ones. One of the many criticisms of these studies is that there is not a good “control group” with which to compare children with craniosynostosis. For example, if you were to test a large group of children who do not have craniosynostosis in order to determine if they have developmental or behavioral problems, you would certainly expect to find that some children had these problems. It is difficult to know for sure if a child with craniosynostosis has a higher chance of having developmental or behavioral issues just because of the craniosynostosis. On the other hand, there may in fact be a higher incidence of these problems in children with craniosynostosis. We still do not know the answers to a number of good questions such as: does surgery help to prevent developmental problems? Are there different problems specific to the different fused sutures? Is the brain less affected in those cases where the skull is less affected? It has been our experience that the vast majority of children with a single sutural synostosis are normal children who just have early closure of one of their sutures.

Does Craniosynostosis Need Treatment?
Why is an operation recommended for those children who have a single sutural synostosis? If one considers all that is known about brain growth and development and reviews all the studies examining raised intracranial pressure, blood flow to the brain, and more recently, brain metabolism, the current final conclusion is that most children need surgical treatment. Surgery is meant to return the skull shape and size to where is should be, which gives the child the best chance to develop normally. If children do not look normal, their appearance can have a profound effect on their personality, their willingness to socially interact with their peers, even their desire to go to school. The most difficult decision for surgeons and parents is for those children who are very mildly affected. If the skull is only slightly involved, it is unlikely that there has been a significant impact on the brain and appearance; then we often recommend not operating. You can get more information in the Treatment section.

Listed below are the four types of single sutural synostoses:



Coronal Craniosynostosis (Unilateral Coronal Synostosis, Plagiocephaly)

“Plagiocephaly” is the term used to describe the shape that results from craniosynostosis, or fusion, of either the right or left sides of the coronal suture. The coronal suture runs across the top of the skull, almost from ear to ear. The soft spot, or fontanel, is located midway between the right and left coronal sutures and is usually absent, or closed, in plagiocephaly. On the side of the skull where the suture has closed, one can typically feel a raised ridge of bone. When viewed from above, the forehead on the fused side is further back than the other side (which typically has slightly overgrown forward, in order to compensate for the brain’s inability to grow on the side that has fused). In looking straight on at the child, the eyebrow on the fused side is usually higher and the upper part of the nose may be off-center, toward the side of the fused suture. Most parents report that their child looks worse when they see their child in a mirror, which may be related to the fact that a mirror flips the image around so the right side becomes the left, etc. Instead of seeing your child as you are used to seeing him or her everyday with the flatness on one side, you now see a “different” child with flatness on the opposite side.

The incidence of plagiocephaly is estimated to be about one in 3500 births. Almost all children affected with plagiocephaly require surgical treatment. The treatment for this condition is discussed in the Treatment section. Also see general information in the Single Sutural Synostoses section.



Sagittal Craniosynostosis (Scaphocephaly)

Scaphocephaly is the term used to describe the shape that results from craniosynostosis, or fusion, of the sagittal suture. This sutures runs from front to back starting at the fontanel (soft spot) at the top of the head, and extends backwards along the middle of the skull. Sometimes the soft spot is absent, or closed. A ridge can be seen, or felt, running along the top of the head in between the right and left halves of the skull. When viewed from above, the skull is wider near the forehead and gets narrower towards the back of the skull (which is the opposite of what is normal: the back of the skull should be wider than the front). When looking straight on at the child’s face, the forehead may seem bigger, and the sides of the skull look narrow.

The incidence of scaphocephaly is one in 2,000 births. It is the most common form of craniosynostosis. Almost all children affected with scaphocephaly require surgical treatment. The treatment for this condition is discussed in the Treatment section. Also see general information in the Single Sutural Synostoses section.



Metopic Craniosynostosis (Trigonocephaly)

Trigonocephaly is the term used to describe the shape that results from craniosynostosis, or fusion, of the metopic suture. The metopic suture runs from the top of the head, at the fontanel or soft spot, down the center of the forehead to the nose. It is different from the other sutures of the skull because it is the only one that normally begins closing in infancy. A ridge can usually be seen running down the center of the forehead and the forehead will look narrow. Often, the eyes are usually spaced closer together than is normal. When viewed from above, the forehead will have a triangular shape, like the bow of a boat.

The incidence of trigonocephaly is somewhere between one in 2,500 to one in 3,500 births. Sometimes it can be very difficult to determine if a child has significant trigonocephaly, or not. Some children who are very mildly affected may not require any treatment. Trigonocephaly has been reported to occur when mothers have taken Valproic Acid (Depakene, Depakote, Convulex) for seizures.

Many children may just have a ridge running down the center of their forehead suggesting that the metopic suture has closed early, however, without producing the triangular-shaped forehead. Only those children who have overt trigonocephaly need treatment. Children with an isolated ridge running down their foreheads do not require surgery (see Publications, Book Chapters #3). The treatment for this condition is discussed in the Treatment section. Also see general information in the Single Sutural Synostoses section.



Lambdoid Craniosynostosis (Posterior Plagiocephaly)

Posterior plagiocephaly is the term used to describe the shape that results from craniosynostosis, or a fusion, of one of the lambdoid sutures. These sutures are located on the back of the skull, with one on the right side and one on the left side, making a shape like an upside down “V.” Usually, only one side fuses shut, but rarely both sides will be closed. When viewed from above, the affected side of the back of the head is flatter than the opposite side. One sign that the lambdoid suture is fused is the presence of a low bump behind the ear, on the same side as the fused suture. Another good way to determine if the lambdoid suture is closed is to check if the ear on the same side as the skull flatness is pulled backwards and, sticks out more. Another sign of lambdoid synostosis is to see if the height of the back of the skull is lower on the flattened side. If the ear is forward on the flat side (with respect to the opposite ear) then a skull deformation should be suspected instead of a fused suture. It is critically important to determine whether or not the child truly has a fused suture because skull deformations almost never need to be surgically treated. There have been a number of articles in the news media about children who were treated surgically for flatness on the back of the head for presumed lambdoid synostosis when, in fact, the child had a deformation and could easily have been treated without surgery (either by doing nothing or by using a headband). Making the diagnosis even more difficult is the fact that on plain skull x-rays, the lambdoid suture can be misdiagnosed as being fused shut. Ideally, the diagnosis of lambdoid synostosis is made by either by an experienced craniofacial surgeon, who can usually tell by an examination alone, or by a CT scan performed at an experienced center. Children, who have lambdoid synostosis and significant flattening of the skull, do require surgery to fix this condition.

The incidence of posterior plagiocephaly is currently unknown and is probably even more rare than one in 50,000 births. The reason that we cannot give an accurate estimate of the incidence of this condition is that it has been over diagnosed in the past. It is probably one of the most rare forms of craniosynostosis treated at busy craniofacial centers. The treatment for this condition is discussed in the Treatment section. Also see general information in the Single Sutural Synostoses section.



Other Synostoses
Sometimes, more than one of these types of single sutural craniosynostosis can occur together in combinations. If both coronal sutures are fused shut, then your child may have a syndrome, sometimes generally referred to as craniofacial dysostosis. For a good starting point, please refer to the sections on Apert, Crouzon, and Pfeiffer syndromes. There are also some even more rare conditions in which multiple sutures are fused in different combinations, sometimes referred to as “complex craniosynostoses.” These rare forms of craniosynostosis are best treated at the busiest national centers.


Treatment


Older Treatments

The earliest treatments for single sutural synostoses were performed by neurosurgeons operating alone. These early procedures, sometimes called “strip craniectomies” or “Pi procedures,” were similar in that they involve removal of the fused suture, in hopes of releasing the restriction to growth (basically, throwing away a section of the skull). However, a number of studies proved that these treatments were almost always unsuccessful. The excised bone usually grows back quickly so that there is no significant change in shape. However, sometimes the skull will grow back incompletely, leaving a permanent open space of unprotected skull. The result is that with these older techniques, the skull shape is never normalized, and sometimes children need to have another operation to either correct the skull shape, or to fill in skull defects that never completely healed in.

Remodeling Procedures

Today, children with a single sutural synostosis usually undergo an evaluation by a craniofacial team prior to deciding on surgical treatment. If needed, surgery is performed by both a craniofacial surgeon and a pediatric neurosurgeon working together (if parents meet a neurosurgeon who recommends operating without a craniofacial surgeon, then I strongly recommend seeking another opinion). Typically, it is the role of the neurosurgeon to safely remove the bone, and the craniofacial surgeon’s job is to rebuild the skull into a normal shape. The specifics of this procedure vary from surgeon to surgeon.

After learning that the “strip procedures” (mentioned above) never completely corrected the abnormal skull shape, craniofacial surgeons recognized that not only is it necessary to remove the bones affected by a fused suture, it is also critical to remodel the skull bones in order to achieve a normal skull shape. These remodeling procedures vary, but there are three basic techniques: 1. Surgery is limited to the area of the skull near the fused suture and is done in a single operation (this is my preference; see “the Dallas Procedure” below). 2. The entire skull is removed, and remodeled in one operation (I think that this bigger operation may add proportionally more of a chance for complications). 3. The skull is treated with two separate operations, one on the back, and then a second one in the front (why double the risks of surgery, when the problem can be fixed with only one operation?).

Endoscopic, or “Minimally Invasive” Procedures

Recently, a few surgical teams have gone back to performing a modification of the older “strip procedure” using an endoscope (operating through a small tube) and then putting a child in a headband for up to a year, in order to mold the desired shape. Some surgeons have used the term “minimally invasive” to describe this procedure. I believe that there is nothing minimally invasive about removing a skull from an infant, and I am concerned that the term “minimally invasive” may be used more as an advertising term, which is misleading. In my opinion, the use of the word “minimal” wrongly suggests to parents that this procedure is a safer operation. In fact, this technique might actually have a slightly higher serious complication rate (and death rate) than a remodeling procedure. One reason for the higher complication rates with “minimally invasive endoscopic” surgery might be because of the limited view that the surgeon has when performing the operation. As a result of the small incisions, the surgeons can only see what is going in by looking through a small tube. This may potentially result in the surgeon not being able to see an area that continues to bleed and should be stopped, or dura (the covering of the brain) that has been accidently torn and needs to be repaired.

In addition to the “minimally invasive” operations probably having a slightly higher complication rate than a remodeling operation, many parents are understandably not happy about the need for their baby to wear a headband for up to a year after the operation. It is important to realize that the endoscopic operation does not significantly correct the abnormal skull shape; it is the head-banding technician who works to mold the skull shape and not the surgeon. The final result is dependant upon the skill of the technician doing the molding, and the ability for the parents to actually keep their child in the headband.

I have seen some children to get a fairly good initial result with an endoscopic repair; however, some children do not end up with a sufficient correction, and must be re-operated on using a remodeling technique. Studies performed at the Dallas Center have shown that if one follows a child’s growth for up to 10 years after correcting sagittal craniosynostosis, growth is not normal and there is a tendency for the skull to go back towards the way it was before the repair (see Publication #25). These new findings suggest that it is very important for the surgeon to over-correct the presenting problem; otherwise, 10 years later, the child may need to undergo a repeat operation. Over-correction is very hard to achieve with an endoscopic repair, and I believe it is likely that we will learn that the need for second operations is going to turn out to be much higher in children repaired endoscopically, once those children treated with this relatively new technique have had a chance to grow up. It is my impression that the younger the child at the time of endoscopic surgery (less than 4-months old), and the less severe the presenting skull shape, the better the result might potentially be, but no one has yet studied this. I believe that parents should only consider the endoscopic technique with very young babies who have sagittal synostosis (because when the child’s skull grows back the way it was, the abnormal skull shape will more likely covered with hair and a second operation would be less likely). Finally, any operation that removes a strip of bone and does not put it back relies upon the ability for the lining of the brain (called the “dura”) to remake new bone to fill in the gaps. If this does not happen completely, then a second operation may likely be needed to reconstruct the missing skull bone.

In conclusion, while the endoscopic procedure is a slightly shorter operation, it is probably a slightly riskier operation, which on average produces a result that is not as good as can be achieved with a remodeling procedure (which at our center, takes about 45 minutes longer in the operating room, and saves months of head banding treatments).

When is the best time to do the operation?

Surgery to correct craniosynostosis can be done at any age. The ideal time is between 4 –10 months, depending on which suture is fused shut.

In favor of the earliest correction are a number of factors:


With early operations the skull is less rigid and easier to shape.
The younger the infant, the better the chance that any small skull defects that might be left at the end of the operation will fill in. After one year of age, babies pretty much lose their ability to make skull bone. So, when operating on an older child, a slightly more extensive operation is necessary in order to fill in all open areas.
The longer a child grows with a fused suture, the more that other areas of the skull may over-expand because the brain cannot grow near the fused suture; this further changes the skull shape in an abnormal way. A study done at our center suggested that for children born with trigonocephaly, surgery before six months of age allowed better growth between the eyes, which is an area abnormally narrowed by the fused metopic suture (see publications #14.)
In favor of operating at a later age are a number of factors:

The operation may be slightly safer. The worst possible complication, following both endoscopic and remodeling procedures, is the baby does not survive the operation. This horrible complication, which is very rare, almost always results from excessive blood loss. As the infant gets bigger, the child has more blood to lose, and there may be a decreasing likelihood that a blood transfusion will be necessary. Surgery before 12 weeks of age is unusual unless the deformity is quite severe. At our center, I routinely give infants a drug called erythropoietin before surgery (see Procrit Information), which typically raises a child’s blood levels to help prevent the need for blood transfusions, hopefully improving the overall safety of the operation (see Publications #19 and #25). I also use a “cell-saver” that sucks up most all of the blood lost during the operation, filters it, and lets us give the baby back it’s own blood. These two different techniques (and many other smaller things that are also done) when combined together make blood transfusions fairly uncommon in my practice. Some research suggests that patients who get blood transfusions are more likely to get infections after surgery.
The other benefit to operating at a later age is that the correction performed is more likely to last a lifetime. Research done in our Dallas Center examined the long-term growth of the skull for over 10 years following correction of sagittal synostosis (see Publications# 25). In one of the first studies of its kind to examine long-term growth after surgical correction, we found that children’s skulls do not grow as they should following the operation (even though the skull shape was completely normalized after surgery); the skull tends to grow back to way it was before the correction. As a result of this research, I have been over-correcting the skull shape for some time, and very few of my patients have needed second operations (less than 2%, thus far). Operating on older children, and over-correcting the skull shape during the operation, probably significantly reduces the chance that a child will need a second operation 10 years or more after the first. Another recent study at our center, yet to be published, shows that the other sutures are similar to the sagittal suture in that growth is not normal after surgery, further supporting the need to over-correct the skull at the time of the operation.


Balancing all of the above factors, it seems that the ideal time to correct craniosynostosis is somewhere between 4 – 10 months, depending on which suture is involved, the severity of the abnormal skull shape, and a number of other factors.

The Dallas Remodeling Procedure.

In my practice, the surgery to correct a single suture craniosynostosis is always performed with both a craniofacial surgeon and a pediatric neurosurgeon, both of whom are present for the entire operation. I believe that having two experienced surgeons always with the child, improves the speed and safety of the operation. In addition, only pediatric anesthesiologist’s with extensive craniofacial surgical experience are typically selected. During the operation, the family is given hourly updates as to their child’s condition. The average correction takes just under four hours from start to finish, although the actual surgical time is much shorter, averaging about an hour and 45 minutes (about half of the total time in the operating room is spent preparing the child for surgery, and then slowly waking the child up). We do not shave any hair for surgery, although many centers still prefer to do so. The surgery is performed though an incision that is made from ear to ear across the very top of the head. Many years ago, I developed a wavy, “zigzag” incision to replace the standard straight-line incision, so that when children get their hair wet it won’t part on a straight-line scar and make the scar obvious (see Publications #10). Recently, I have shortened the length of this incision (starting it well above the ears) so that the scar will be even less noticeable.

The goal of the operation is to remove the areas of skull that were affected by the fused suture and rebuild the skull into a normal shape (which is slightly over-corrected to compensate for the abnormal growth that that will occur). In order to rebuild the skull, it is necessary to somehow hold the bones together, so that they can heal. Most surgeons use plates and screws to set the bones, however, I prefer to use dissolving stitches for a number of reasons. To begin with, after operating on children who had initially undergone surgery at other centers, we discovered that when other surgeons used metal plates and screws to hold the skull bones together, with the subsequent growth of the skull these plates will end up on the inside of the skull with the screws poking into the brain (see Publications #12). Studies elsewhere have noted the same thing happens with some of the dissolving plates and screws: they can also end up inside the skull against the brain before they dissolve. While I am not aware of any cases in which this has caused a problem I have decided to use dissolving stitches to avoid any potential problems (see Publications #20). I have also found that when plates and screws do dissolve, they can end up leaving a weak space inside of the skull bones that can make additional operations (if needed) much more technically difficult. For this reason, I feel fairly strongly that they should be avoided in children with syndromes (who are most likely to need additional operations) such as Apert, Crouzon and Pfeiffer, etc. Finally, in a small percentage of cases, dissolving plates and screws will melt into a liquid that collects in a pocket under the skin, before it opens a hole on the face or scalp and drains out. Given all these potential problems, I am convinced that while it is technically more difficult to use dissolving sutures to rebuild a skull instead of using plates and screws, it is what I would want for my own child.

At the end of the operation, we close the scalp with dissolving stitches. We never use metal staples or non-dissolving sutures, as these can hurt when they are removed. We also do not put any bandages on the child, and do not use any drainage tubes (I believe that these tubes make the recovery actually slightly more complicated). Instead, the child is given a shampoo before we leave the room and the hair is combed over the incision. Many surgeons do like to use head wrap bandages; this is not “wrong” in my opinion, but these head wraps are unnecessary.

The child usually will spend one night in the pediatric intensive care unit and are transferred to the floor the following day. While most of my patients might be ready to leave the hospital in about 24 hours, I recommend all stay for at least two nights total in the hospital. The risks of the surgery are very small at the most experienced centers. In a published report (see Publications #15) looking at a combined experience with our center and one other center, we found that no infections occurred in infants undergoing operations for craniosynostosis (although this is certainly possible).

When craniosynostosis corrections are performed on older children, they tell us that they feel almost no pain (they do say that their skin feels “tight” with the swelling that occurs). Nevertheless, we have a policy of playing soft music in the child’s room after surgery because a study showed that soft music relaxes children, and reduces the amount of pain that a child will report feeling after the operation (it also reduces the amount of pain medicine needed).