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Interviewer: Three types of surgery can be used to treat craniosynostosis, which one depends on a few different factors. If the synostosis is caught early enough, the newer endoscopic procedure can be used.

Pediatric plastic surgeon Dr. Faizi Siddiqi and pediatric neurosurgeon Dr. John Kestle are here to discuss the three different types of surgery for a synostosis and why they would consider one over the other. So, first of all, how early does a child need to see you for the less invasive endoscopic procedure to be an option?

Dr. Kestle: So the endoscopic method we've been doing since '07, and we've found that the best time to do it is usually between two and three months of age. So we have to see the patient before that and the earlier the better. The surgery is done under a general anesthetic, and the surgery typically lasts about two hours, and most of them spend one night and can go home the next day. Occasionally, they need two nights. It's done with, for example, sagittal synostosis with two incisions -- one just behind the soft spot and one toward the back of the head. And then we work under the skin to remove the fused bone.

Removing the fused bone doesn't really change the shape much at all immediately. It just releases the bone. And then about two weeks after the surgery, they start wearing a special helmet that's designed for surgical patients, that is a little snug front to back and a little loose on the sides, and it just guides the growth so that as the baby's head growing, it's taking on a more normal shape. They wear the helmet for about six months, some shorter, some longer, and we just monitor the growth pattern and make that decision. And they'll go through several custom-made helmets over the course of the treatment as they grow.

Interviewer: Generally, I'd imagine a less invasive procedure is always better. Why is this procedure better than say the traditional treatments?

Dr. Kestle: Well, it's got a shorter hospital stay, a much lower rate of blood transfusion. It's a lot less swelling associated with the surgery. It's easier on the babies, and the results are at least as good.

Interviewer: Is there a reason, other than age, why a child would not be eligible for the endoscopic surgery?

Dr. Kestle: We don't often do it in children that have syndromes where they might have multiple malformations in other parts of the body, such as Crouzon syndrome or Apert syndrome. And in addition to those other malformations, they have synostosis. Those children usually need the more traditional, bigger surgery. But any child that has one suture fused is a great candidate for the smaller surgery.

Interviewer: Dr. Kestle, you're a neurosurgeon. So you handle that part of the procedure?

Dr. Kestle: Yes.

Interviewer: Okay. And then Dr. Siddiqi, you're a pediatric plastic surgeon. With the endoscopic procedure, both of you are in the operating room at the same time. Just kind of walk me through how the surgery goes. Dr. Siddiqi, you start the surgery.

Dr. Siddiqi: So we're in the OR together. It's a team approach. So once the anesthesia team have completed their part, which is getting the baby asleep and making sure the IVs are put in and everything is safe to proceed, that's when we position the baby for surgery. I would make the initial incisions. For example, for sagittal synostosis, we make two incisions on the top of the scalp. Again, that's one of the advantages of doing it this way versus the bigger procedure because you have two small incisions. Through those incisions, we expose the area that we want to operate on, which is that fused sagittal suture. And once everything's exposed and visible, then Dr. Kestle would take over.

Dr. Kestle: What we do is remove a little bit of bone under each incision. And that allows us to get underneath the bone. Underneath the bone is a layer called the dura, which is a covering layer over the brain. It's kind of like leather, like a thin leather. And we use the endoscope to separate that layer from the bone, and that allows us to safely cut the bone and remove it. Once the bone is removed, we look at the dura and make sure it's okay. We stop any little bits of bleeding, but there usually isn't much. And we check the bone edges, which sometimes ooze, and make sure that any bleeding is stopped. And then at that point, Dr. Siddiqi and his team continue working.

Dr. Siddiqi: Yeah. So we take out or remove additional segments of bone. There are these little triangles we take out, about four them. Again, afterwards, we make sure that the bone edges are, you know, clean. They're not bleeding. Again, that's one of the other advantages to doing it this way. The blood loss is quite small, minimal compared to the traditional way. Most of the time, it's maybe 10 or 15 milliliters of blood. So once those triangles are removed, then we close the incisions. Then the anesthesia team takes over, and the baby's, you know, woken up and then taken to the recovery room.

Interviewer: What does the recovery look like then for a child? And, you know, what kind of outcomes can parents expect?

Dr. Kestle: With the small surgery, they don't need to go to the intensive care unit. They stay in the hospital in a regular room, and the parents can stay with them. The vast majority of those children are here for one night. Occasionally, they need two nights. The criteria for going home are pain control and feeding. They get some swelling toward the back of the head, that gradually goes down over the first week at home. Stitches dissolve on their own. And within a day or two, they're back to their usual self as far as feeding and behavior goes.

Interviewer: And Dr. Siddiqi, how long does it take for the head then to regain more of what would be considered a normal shape?

Dr. Siddiqi: Yeah. So as Dr. Kestle mentioned earlier on, the shape doesn't change after the surgery, right after. It's once they're in the helmet. The helmet is critical for reshaping the head. And typically, they're in the helmet usually two to three weeks after the surgery. It's a custom helmet. You know, it just guides the growth of the head, and over the ensuing, you know, three to six months, we have a more normal head shape. And hopefully, after six months of helmeting, that's all they need.

Interviewer: And the incisions that were talked about out in the endoscopic surgery, are those visible or are those in the hairline?

Dr. Siddiqi: You know, they're in the hairline. And again, another advantage to doing it this way is the incisions are on the top of the scalp, the head, and those scars heal very nicely. They're quite thin and they're barely perceptible. You only notice them when the hair gets wet. Again, with the bigger procedure, you have a much bigger incision from ear to ear, which is much more noticeable.

Interviewer: Let's talk about the more traditional procedures in the event that a parent is in a situation where their child is older than six months old or there's other reasons why they might have to have that. What are the two procedures, and can you explain those a little bit?

Dr. Siddiqi: Yeah. So sometimes, you know, we do see kids who are, you know, two, three months old and they're eligible for the smaller procedure, but for various reasons, let's say they live out of state or they don't want to do the helmeting, they would like to do the traditional, what's called cranial vault reconstruction with orbital advancement. So then we would wait until they're 10 to 12 months of age to do that procedure. Essentially, that's a much more involved procedure, but it's a procedure that's, you know, well described. People have been doing it for, you know, 30, 40 years. You know, the results that you get are comparable to the endoscopic procedure, but, again, it's how you get there.

So with this procedure, you have to expose the entire skull. So that means an ear-to-ear incision through the top of the scalp. I would expose that, mark out where I want Dr. Kestle to make the cuts and remove the segments of bone that we want to reconstruct and reshape. Then Dr. Kestle would remove those pieces of bone, make sure that the lining of the brain is okay, make sure everything is okay. Then I would reshape all those bones and reconstruct the skull in a more normal configuration, and everything is stabilized with plates and screws. And these are resorbable plates and screws. They dissolve in about a years' time. So we put everything back together and close the scalp. That's a four or five-hour process. Much more blood loss than with the endoscopic procedure.

They typically would go to the intensive care unit for one night, and they typically would be in the hospital three or four nights. Oftentimes there's quite a bit of swelling. The eyes can get swollen shut, and it would take maybe 10 days to 2 weeks for that swelling to go down.

Again, the advantage is you don't need a helmet. It's all done in one stage. You know, the compromise is that it's a much bigger operation.

Interviewer: If parents are evaluating a center or physicians to do this procedure, what advice would you give to them to, you know, pick out the best place for them?

Dr. Kestle: I think it's a procedure that is usually done very safely, and children do very well and go home quickly. We are exposing the layer over the brain, and there is a potential for bleeding. And so I think that experience matters. And I think that you do want to be treated by people who do this a lot and people who can handle problems, which are rare, but if they arise, they need to be dealt with appropriately. So I think it's a big advantage to being treated by people who have experience with this, who are in a children's hospital with pediatric-trained specialists, including anesthesia and a pediatric intensive care unit if they need that.

Interviewer: And you mentioned a third procedure, a cranial vault distraction, when might that be used?

Dr. Kestle: So there are some children where their brain is in trouble or potentially in trouble because they have presented very late or they have multiple sutures that are closed. And in that situation, we want to make the skull bigger to give the brain room to grow. Probably the best way to do that these days is a procedure called distraction, where some implants are inserted and then the skull is gradually expanded over time.

Dr. Siddiqi: You know, with cranial vault distraction, again, the idea is to give the brain as much room as we can because of the fact that more than one suture is fused. And the way that's done is I would ask Dr. Kestle to make some cuts on the bones. And then I would put these little devices, they're called distractors, on either side of the cuts. And then three days after surgery, we would have the family start turning those distractor devices. Typically, it would be total of one millimeter a day. So over the ensuing three to four or five or six weeks, the bones are slowly being separated. And what happens is that, as they're separated, there's new bone being formed in the gap. And over time, that new bone will solidify. So we're not only expanding the volume for the brain, we're also creating new bone. This is really the only way we can expand the brain to this degree using these devices. We couldn't do it as a single-stage procedure.

Interviewer: Between the two procedures, the endoscopic procedure, the less invasive, and the cranial vault reconstruction, are there any tangible differences and outcomes or how the head is going to look or anything like that?

Dr. Siddiqi: I would say like the overall head shape is probably going to be comparable, just the head shape itself. But again, as I said, it's how do you get there? You know, how long does it take? What are the risks involved? In terms of the shape itself, with the endoscopic procedure, overall the head feels and looks quite smooth at the end of the day when everything is healed. Whereas with the bigger procedure, you know, we're taking all the bones out or in multiple pieces, putting it back together. So when everything is healed in a year, two, three years' time, you do feel some irregularities over where the bones are joined together. But overall, the head shape is probably comparable. But, you know, I think you get an overall smoother head shape. And I think it probably looks a little bit better as well.

Dr. Kestle: And obviously, the scar is different as well. In the endoscopic, there's two scars on the top of the head that hide really well. And the bigger surgery has an ear-to-ear incision, which also usually heals really well. But as people age and start to lose hair, it's a lot more obvious.

Interviewer: Craniosynostosis. It's a condition that causes a newborn's head to be misshapen. And it should be treated. To learn more about the condition, how to identify it, and why it needs to be treated, we've got Dr. John Kestle, he's a pediatric neurosurgeon, and Dr. Faizi Siddiqi, he's a pediatric plastic surgeon. And they are experts. They do the surgery that actually treats craniosynostosis.

So let's start with the first question, which is very basic. Dr. Kestle, what is craniosynostosis?

Dr. Kestle: So it's a condition where the bones that are normally separate are fused. And it restricts growth and creates abnormal head shapes. The pattern is usually present at birth or very soon after birth. That's different from the benign conditions where the head shape gets distorted because the baby is laying on one side. Those babies have a normal shape in the beginning and their head shape gets distorted over the first couple of months of life because they're lying on one side.

Interviewer: How does a parent generally find out that their child has craniosynostosis or synostosis?

Dr. Siddiqi: When they're born, they're usually told that after delivery, as Dr. Kestle mentioned, the head is going to be a little misshapen from the birthing process. And that usually corrects within two to three weeks. If that doesn't correct, then they're kind of suspicious and they visit their pediatrician. And then hopefully that's when they're referred for further evaluation by us.

Interviewer: And then what does that head shape look like? We do have a link to a pamphlet that you have that can help a parent. But just describe it briefly.

Dr. Kestle: So the typical head shapes, number one, most common is sagittal synostosis. It makes the head long and narrow, and the forehead and the back of the head kind of stick out. And the back of the head is narrower than the middle of the head. You can see those features when you look down from above.

Probably the second most common type is metopic synostosis. And that's when the suture down the forehead closes early and the forehead looks like the bow of a boat, or a triangle.

The other two types are less common. One is coronal synostosis, and that misshapes one side of the forehead so the forehead is pulled back, and the nose is sometimes crooked, and the eye socket on that side is usually a little bit elevated. And then the very rare one is the lambdoid synostosis, where the back of the head is flat on one side and the ear tends to be pulled back toward the flat side.

Interviewer: So how is it diagnosed then? So a parent recognizes that their child might have a misshapen head, they're concerned, they would go to a pediatrician first?

Dr. Siddiqi: Certainly they visit with their pediatrician and then they're referred to our synostosis clinic for further evaluation.

Interviewer: Okay. The pediatrician doesn't do any sort of imaging or anything like that generally?

Dr. Siddiqi: Sometimes they do. Oftentimes they don't because they don't want to subject the child to a CT scan unless they've seen a specialist and they're confident of the diagnosis. So we would see those kids in the clinic. Most of the time, it's a clinical diagnosis.

Interviewer: Meaning it's just visual, you're visually confirming it?

Dr. Siddiqi: Yeah. But once we decide it is and we talk about surgery, then we would want to get a CT scan, generally speaking, to be definitive about the diagnosis. And the scan also gives us information about the brain, which is helpful as well.

Interviewer: Why do you choose a CT scan over, say, an X-ray or some other sort of imaging?

Dr. Siddiqi: A CT scan gives much more detail of not only the bones, but, as I said, also the brain as well.

Dr. Kestle: The X-ray is very unreliable in making the diagnosis. And the CT scan has been changed over the years so the dose of radiation that's received is lower and lower. So now it's a very reasonable thing to do. It gives excellent anatomy, helps us with planning a surgery, makes the surgery safer.

Interviewer: And, Dr. Siddiqi, when do you generally like to treat a synostosis then?

Dr. Siddiqi: So if we see a baby with, let's say, sagittal synostosis that comes in at 2 to 3 weeks of age, then we've seen that the optimal time for surgery would be somewhere around 3 months of age. Between 2 and 4 months of age. That's why we stress the importance of early referral.

And the reason for that is if we can get the kids in by that time, we can get the surgery done, and then we can get them in the helmet and the duration of a helmeting would be as short as six months. So if we see these kids later on, the helmeting duration is much longer.

Interviewer: And, Dr. Kestle, if a child doesn't receive treatment for a synostosis, what could be the potential outcome then?

Dr. Kestle: So the natural history is that the shape will stay the same or get progressively worse because everything is growing except the fused suture. So number one, it's an issue of shape and appearance.

Number two, there are potential effects on the brain if it's left untreated. There's an incidence around 15%, maybe 20%, in the kids with sagittal synostosis that the brain growth will be restricted. And that can lead to brain problems, chronic headache, possibly visual problems.

With the other types of synostoses, that number is a little higher. And so it's nearly impossible to predict which baby with synostosis is going to get into those brain problems later. But that incidence of raised pressure is enough that we worry about leaving it alone.

Occasionally, we'll see an older child who had a CT scan for another reason, like maybe a concussion, and we identify a fused suture. But their shape is normal, and they're healthy, and their brain is developing normally. That's the situation where we might just follow them. But the baby that has the abnormal shape soon after birth is the ones where we recommend treatment.

Dr. Siddiqi: I would just add that . . . echo what Dr. Kestle said. The two indications are the shape and the risk of pressure on the brain. But the shape is important. A lot of families ask, or even other providers say, "Is this cosmetic?" It's not cosmetic. Cosmetic means, by definition, it's normal and you're making it better. It's not normal to have craniosynostosis. I just wanted to make that clear.

Dr. Kestle: I just want to really emphasize that it's visual. It's the shape that makes the diagnosis. So much so that what we do for almost every patient is have the family send photos. And we can pretty much make the diagnosis from the photographs and then decide if they need additional testing, how quickly we need to see them, and so on. But it's really a visual inspection of the head shape that tells you the diagnosis 90-plus percent of the time.

Interviewer: So really at 3 weeks, if a parent suspects that their child might have a synostosis, they should get in contact with a couple of specialists or a specialty center such as yourselves. You would encourage them to do that as quickly as possible. Is time really of the essence?

Dr. Kestle: For sure. And we can make plans based on photos, and we can see them in the clinic and talk about it, and then targeting between 2 and 3 months for corrective surgery.