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Grand Rounds-Endoscopic Management of Metopic and Lambdoid Synostosis

David Jimenez and Cormac Maher

November 14, 2013

Transcript

- Hello and welcome to another edition of WNS operative grand rounds. And today we're very fortunate again to be joined by Dr. David Jimenez, who is the professor and chairman of neurosurgery at University of Texas, San Antonio. And as all of you know, Dr. Jemenez is really the creator of many of the surgical procedures that we'll see today and has been writing extensively on these topics now for many years. So we're very privileged again, to be joined by him as he takes us through the next set of craniosynostosis topics here in part two of our craniosynostosis series. And this time we'll be focusing on metopic and lambdoid synostosis. So Dr. Jemenez, thank you again.

- All right, thank you very much. I will also like to disclose that this work has been done in conjunction with that Doctor Barone, the plastic surgeon whom I've worked with for the past 27 years and has come up with a lot of the inspiration for what we're doing. So today I would like to talk to you about the management of both metopic and lambdoid synostosis. We've already talked about sagittal and coronal, and in those videos I've described the instrumentation and some of the basic principles. I'll concentrate on some of the results. We've operated so far on a total 127 metopic synostoses. So it's a pretty good number of patients over the past 17 years. In this case where you can see here is that the patients typically do have severe trigonocephaly. I want to make a point that we do not operate on metopic ridges.

- Thank you very much. When you say you don't operate on metopic ridges, that of course, is something that we would all subscribe to, but how, when does a metopic ridge become metopic synostosis for you? Are there objective criteria that you look at for making that surgical determination?

- Well, I think technically all metopic ridges are metopic craniosynostosis 'cause then that is what happens. The question in my mind, or at least in our mind is when does that take place? If metopic synostosis develops later in life, meaning, you know, five, six, seven, eight, months of age on a child that otherwise was normal, then it becomes a metopic ridge, but clinically I don't think is significant. All the patient has, is a little bump somewhere up in this area. But when you look at the child from above, the shape of the forehead is not trigonocephalic, it's nice and round. When you look at their eyes, they're not hypoteloric. They don't look pinched in. And other than perhaps a little visible ridge, you have a totally normal child. Where we believe that the clinically significant metopic synostosis is when most of these happen in utero anyway, and the child is born with the synostosis. And these kids show up like you see that this child in this slide. Significant hypotelorism, the eyes are moved in the middle, significant trigonocephaly, and quite a bit of compression on the frontal bones. This illustration here is to let you know that the concept is simply to do a metopic suturectomy extending from the anterior fontanelle all the way down to the nasal frontal suture, but we must reach the nasal frontal suture. Leaving any bone up in here will defeat the purpose of the operation. Following, and this is about the location of the incisions. We make it right behind the hairline and usually in front of the interior fontanelle. It usually measures about two centimeters in size in length. And then this is followed by the placement of specially custom made orthotic helmets, which again will put pressure right here, some pressure here, pressure, not really pressure, but containment, and then leave an extra space right in this area, both on the front side, which then allows the brain to grow in this direction, fill in and obtain a normal round forehead. Now, I would like to go into a surgical video to show you the exact operation and how it is done. This video demonstrates, first of all, we'll start with the instrumentation. As I mentioned previously, we'd like to keep things simple. And almost all the we use is contained in a Mayo stand right there. This is an electric rasper that has become very useful in smoothing of the edges of the osteotomies. We use some cottonoids, some gel foam, some thrombin, some surgiflow, a craniotome to make the burr hole. And then the some carrots and rongeurs. I would say that perhaps a very critical piece that we use are these things. These are made by AkroMed. These are, they look like pituitary rongeurs. Came from an orthopedic tray. These are bone cutting rongeurs. These will cut any kind of bone in a very efficient fashion. So here's the child. Again, you can see this is not metopic ridge. You can see the child has significant bilateral proptosis, trigonocephaly. Like I mentioned to you before monopolar electrocautery set at 1515, blend one is used to make the incision to violate the pericranium as bleeding will take place. Pericranium is left in place. We palpated there right about here where the anterior fontanelle is. Once this is exposed, we do a little bit of subgaleal dissection to make sure that the galea is separated all the way back to anterior fontanelle. And that can be done with a rhinoplasty lighter retractor. We now pay attention more anteriorly, do a little bit of dissection using monopolar electrocautery under direct visualization. This dissection can be then taken all the way down as far as we can. Again, using the rhinoplasty lighter retractor. You can also use brain ribbons and a zero degree endoscope. And what we see is that when we're done, you can place a brain ribbon all the way down to the nasal frontal suture. That's all the dissection that's necessary. We then go on to do a burr hole, just like we did before. We score the pericranium. Use of pediatric craniotome right over the sagittal sinus, right over the metopic suture. We have not had any dural tears, perforations, or any problems doing this. You want to keep it right in the midline, because like with the coronal synostosis, you don't want to make a really wide osteotomy or you end up, you may end up having bony defects. Once we do that, we remove the remnant bone that's left there. And then a number one Penfield is used to go back and free the little bit of bone that's left between the anterior fontanelle and that osteomy. Now we know now that it's free. So it was very easy to take a pair of Mayo scissors and just do a little wedge osteotomy and cut that off. As you can see right there, we don't need anything fancy, anything more than pair of curved Mayo scissors. And that's all that's necessary. If needed, it can be enlarged a little bit with Leksell rongeurs if necessary. Once again, I must stress that we have to have the osteotomy go to anterior fontanelle. Now comes the challenging part. Now to tell you that of all the sutures that we do, perhaps these metopics are the most challenging. And the problem is particularly because we are working through a very channel, which is only six millimeters in width. What we'd like to do now is to enlarge the burr hole in this fashion, using the carrots and rongeurs. These kids tend to have thick bone and oftentimes where the suture is stenosed, there is a big fat queue which tends to bleed. So oftentimes what we do is we make sections of bone removal. We go and we cauterize. We're now, we've gone, here's the scalp, above here. Here's the dura. We've done a wedge osteotomy about halfway down the forehead. You can see here that the bone oozing as typically does. This is the instrument that I was telling you about. This is the key piece. So we do a wedge osteotomy to about this point right here and then from here down the rest of it is done in a piecemeal fashion using these bone rongeurs. A good idea is that as you go, as you keep going down, you keep cauterizing because if not a thick bone can ooze quite a bit and it makes visualization hard to do. Oftentimes in this section right here, there are a number of significant size perforating veins tend to bleed and they can cause quite a bit of bleeding. So what we've done is we all the patients have a precordial doppler placed and when the bleeding starts to get a little more profuse, what we do is we tend to elevate the head of the bed, listening for venous air embolisms. This is a key instrument. This is the bipolar endoscopic bipolar. This, you can't use the regular bipolars here because as you try to open them, they won't open in the very small osteotomy. So here, one of those perforating veins that I was telling you about right here, you can see them or with the forceps, you can get perfect access. You can take yours endoscope right to the vessel, put your suction down and cauterize. Now we've gone all the way down to nasal frontal suture here. You can see the dura attaching to this and to make sure that we have completely finalized the opening, we take an osteotome and tap all the way down and make sure that the nasal frontal suture is splayed open. And then we can move the osteotome side to side and you can see the bone is free. This is a perfect example. So here here's the nasal frontal suture way down here. And now you can see how we're moving the bone and the bone is completely freed. The left orbit is here, right orbit is here.

- You mentioned the precordial doppler. Do you use that for other synostosis cases or just the metopic because of the bridging veins upfront?

- We use the precordial doppler in all cases.

- All cases.

- Yes, I mean, it's so simple to do, and if you have to elevate the head up or when we're washing or irrigating, we we've been monitoring and all the cases, and we haven't had a real problem with it. So it's good to use. So, as I was saying, what happens if you have someone who has a forehead like this and you make an osteotomy and you leave it alone, you're going to end up with two significant poise of bone. So we used a rasper that the plastic surgeons often use to rasp the nose when they're doing rhinoplasties and you take the edge and you basically smooth it out so instead of ending with corners like this that are pointed, they're nice and round. Smooth it out, some surgiflow. Our incisions are closed with 4-0 monocryl galeal sutures one single Steri-Strip, and that is really the extent of the operation. So the challenge I would say, really is just getting down here. Once, once you get down here, you have control, results are pretty good. We can go onto the rest of the presentation. So just to review this briefly, 74 males, 27 females, mean age of about four months. We've done them as young as three weeks. Early on when we're trying to figure out what would be the right cut off, we did a kid who was a year old, but didn't work all that well. I'm going to go through some of the results to show you an average blood loss of that 40 cc. So this higher than the rest of the cases. We've had some significant losses up to 250, and this is a multiple suture synostosis. About a 7% transfusion, oh I'm sorry, if you look at the percent of blood volume loss, about 7%. We've had one intraoperative, blood transfusion and six postoperative blood transfusion. So we still have about 6% transfusion rate, which compares better than 100% with traditional surgery.

- So what is your criteria for a postoperative transfusion? What's your hematocrit that you look at?

- We were very liberal on that. I would say, as long as the patient is saturating well, if the patient has good saturation, if the patient has, is not significantly tachycardic and looks okay and doing fine, we'll tolerate hematocrits of 18, 17. And because we know there's no further blood loss. When we're done, there is no more blood loss and the kids will make it up on their own. The ones that we've had to transfuse, usually got down below 15, or kids who were maybe in the high teens but were significantly tachycardic, or they weren't oxygenating and all that well. Those were the ones that we transfused. The average width of the osteotomy is six millimeters. Length about 10 centimeters, which is the distance between the anterior fontanelle and the nasal frontal suture. We had two class two air embolisms where you can hear 'em. You can hear the girdling and all that, but no significant effect, no injuries to the sinus, no dural tears. Obviously no visual problems or deficits 'cause we don't mess around with the eyes. We did have two CSF leaks, and these were not necessarily dural tears, but the peels were so prominent that when we removed them, CSF began leaking. They were treated simply with a lumbar drain or spinal taps, no need to go back and re-operate. Early on we had, when we made wide openings, we had a couple spots. Two patients who had skin irritations were treated with antibiotic. No deaths. This, I want to address this point, which I think is important that hypotelorism whether you think is true hypotelorism or pseudo hypotelorism, but it is there a significant correction with lateral orbital displacement. So here's an example, the child that I showed you earlier, you can see the eyes are very close together. The epicanthal folds almost filling the entire field and it's the child for surgery. And he was a child eight years of age. You can see that the eyes have really come apart and we didn't do anything to the orbits. There was no hypotelorism surgery. All of that work was done by the brain. And it was because with the health of the helmet, doing the surgery early, letting the brain do what it's supposed to do. Then here's a child at eight years of age with the forehead that does not look like that at all. I'll show you some clinical cases. He's a three month old male, 46 minute surgery, 15 blood loss went home next day. Again, again, you can see that the look the trigonocephaly, big significant mid-line ridge there. And as time goes on, you can see the forehead started to change. You see the helmet is putting pressure right here and this area is now starting to come out as early as two months of age. And then two years of age, you can see the difference here. You can see a forehead that is now normal. And if you look at the kids before and after, you can see how they eyes have indeed moved out laterally, and the child looks normal. He's another kid. This kid was a five and a half months, 45 cc blood loss. Again, you can see, this is not your typical metopic ridge. This is significant trigonocephaly hypotelorism. And now what we see here at two months of age, early on, you can see the, that the point begins to expand as the helmet keeps pressure on that area. The forehead begins to come out laterally. Five months, the child continues to improve. And here the kid's at two years of age looking, what I think is it's normal. So before surgery, two months, five months and five months, the kid that you cannot see a scar, and that looks absolutely normal. With look at the eyes, how they move down, all done internally with that little incision in that early operation. So it seems to me, and it seems to Dr. Barone as well with her large experience of in key plastic surgery eyes that although these surgeries are tough to do, they're challenging, that metopics are giving us absolutely wonderful results. That we've been very pleased with the outcomes as you can see from the few slides that I'll show you. I'll show you more. 2.7 month old child. You can see the kid has that look. Now there's a group of patients. I want you to pay attention to this, that their forehead is slanted back significantly. It's almost like somebody sat on her forehead and they have the significant compensatory growth posteriorly. And she again has trigonocephaly. And look what happens over time. At two months of age, the forehead begins to correct very nicely. Four months of age already look at the difference that is taking place on the shape of a forehead. At a year, two and a half years. And here is the child at four years. So if you were to look before surgery and after surgery, look at that forehead. And this was all done again through a single incision surgery, which is took, you know, over an hour and a kid who now had four years of age, and she's getting to go to school, looks absolutely normal with no scars. Here's another kid, has kind of, this is another one that's similar phenotype, flattened forehead, almost squashed in there. And as you can see over time, the child continues to improve at a year later, three years later, the child has now a round normal forehead. Think I have a couple more here. This is a long-term follow-up on this kid who's just a few months old. Nine years later, again, you can see the difference in the forehead in a kid who had nine years of age is mentally normal, doing very well in school and who looks normal as well. So here she is before and after, before and after, before and after. Notice the proptosis. Now see how the orbital rims have come forward? And we never did anything to the orbital rims. We never did an advancement, which is what we typically do when you do a CVR. This is all done internally, and you can't see your scars. We would like to, I like to go onto the lambdoid synostosis, unless you have any questions.

- Dr. Jiminez, you've shown us lots of wonderful pictures, pre-op, post-opioid, one thing we're not seeing, which I think is remarkable is no CT scans, no x-rays. Is there a role for that in your practice, or is it entirely visual diagnosis now?

- So because of the short time that we have here, I have not shown any of that. Early on, we did used to get CT scans and I have a number of scans showing the major differences before and after. But as we've learned more and more of how much radiation kids are getting, I really, I really shied away from doing cat scans in children anymore. We do extensive anthropometric measurements with the scans that we use, the machines, the infrared scanner. We do all kinds of measurements, which again, this is beyond the scope of this talk. But we, we do the asymmetry all kinds of different measurements. And so we can, we have the numbers to document besides the pictures that we do have. We do not get CT scans for that, for that reason.

- I think that's great. So given the now known risks of radiation, you've gotten away from, from CT scanning in these patients. Would only get it if there's red flags or neurological concerns and maybe get an MRI scan, but a CT and x-ray is not a routine part of the workup anymore in your practice.

- No, it hasn't been for years. I mean, you know, the diagnosis is really a clinical diagnosis. I mean, somebody who knows what they're looking at can make diagnosis of block away without needing anything else. I hate to see these kids with this massive 3D reconstructions for positional plagiocephaly. And if we do need to, if we have concerns about it, we'll just get a simple protocol MR, which can be done in our hospital. We have a protocol that we can do an MRI of a brain in a kid and in about five minutes or so. And it shows us what we need.

- Thank you.

- Okay, let's finish with lambdoid synostosis. Fortunately, this is not a real common problem. And, but we've also addressed this as it presents in our practice. We've only really had, I'm sorry, this is a mistake here. This is 15 not 51. A total 23 patients in the period from 1997 till now. So approximately one patient a year, which is exactly what we would expect for lambdoid synostosis. Most of them were females, ages ranged from a little under a month to about a year. Technique that we decided to use for this is the same concept as all the other sutures are open. And as we were saying before, we don't get the scans but almost every one of these patients show up in our office with a scan, so we make sure that all the sutures are open only the lambdoid. Now we know that a classical lambdois synostosis looks like. So the idea is really to recreate a new lambdoid suture. One way of doing that is simply to make two incisions. This is the, in this case, this would be a patient with a right lambdoid synostosis. So they would make, make an incision right next to the lambda and right next to the sterion. Pretty much like that. They don't need to be that wide. And this is a example of a patient. And we mark in surgery. In order to figure out where this is, we can either get a, I get a simple fluoroscopy shot, used an X spot or, or some kind of in this case, a little clip to make sure that we get the correct position for where the incisions are going to be made. And then the idea is like on the other sutures, we wanted to do a subgaleal dissection from one end to the other Make burr holes, get underneath. And then once the bone is free from the underlying dura and the overlying scalp simply cut and remove. Get above, get underneath and then do a simple strip craniectomy. You do have to make sure, though, that you go from lambda to a sterion. So here's an example of the X that I was telling you about. Just put a little stick on, get a plain x-ray Sometimes you have to move them up a little higher or lower depending, and then that'll show you where your incisions need to be made. In this case, this kid, we know that lambdoid synostosis was primarily from here to here, and then this area was still open and this area was open here. Let me go on to the video now that shows the operation. So in this case, can the child is we place the child in the cerebellar horseshoe with their head, literally just turned to the contralateral side. This is the incision. So the lambdoid's about in this area right here. Like we've shown you before a monopolar electric cautery, 15, 15 blend wand does a beautiful job. Bloodless fascia, no bleeding down to the pericranium through the galea. We do the same thing right next to the asterion on the involved side. You can see that this ear has been pushed down and prominent mastoid. In a bloodless fascia, an incision is made. This area here has to have a little more soft tissue underneath some of the muscles in this area you need to dissect, as you can see right there. But as long as you just go straight down onto the bone, you'll be fine. So typically what we use is a rigid endoscope. These are mostly zero degrees. Like right now this is a zero degree endoscope for a good view. We're doing dissection of the galea below towards the asterion. Making sure we don't violate the galea and get bleeding. We're now looking in with the rhinoplasty lighter retractor, we get great visualization. So we were all the way down to where we need to be. We have now have done that subgaleal dissection again, from lambda to asterion, as you can see here by the brain ribbon being underneath. Now, as far as the width of this craniectomy, again, it doesn't need to be that wide. Perhaps a centimeter on most because that brain is going to increase it as time goes on. Once again, in a similar fashion, we'll begin using a pediatric craiotome seven millimeters, right at the level of the incision. So you can see right there. Little bit of gel foam, and subgaleal dissection is undertaken in a similar fashion. So we've now elevated the scalp. This is the brain ribbon. We've now made the opening. Now we're getting under the bone and you can see here. So if you're right handed in my case, I used the endoscope with my left hand and the suction on my right, as it is serves at dissector, as well as to keep any bleeding, you can see the ridge right there. This is the stenosed lambdoid suture right there. Instead of it be an open, you can see this prominent ridge right with a pointer is all the way across right there. You can see peel extending down, and then now it's open again. So you can see that when the suture is open, you can see the sharpie fibers and dura going right into it. So as long as you stay within the closed area, the likelihood of tearing the dura is really minimal on this. You do a long maneuver. So now we want to make sure that the incision has come all the way to they asterion. And we'll take further pieces of bone using the rongeurs. A little bit of gel foam with a cottonoid usually it does a pretty good job. Now in a similar fashion, we can use either the bone cutting scissors, or we can use a pair of curve, sharp curve, Mayo scissors. Once the, once the dura is free, we simply cut one osteotomy on one side, we do the osteotomy on the other, and then remove a strip of bone. I have to tell you that this lamboids are kind of challenging to treat because they have a significant changes with the skull base and, they are a little bit challenging and they do take some time, but still considering the risks involved in doing the traditional surgery and Dr. Barone and I came up with the technique of what we, what we call the sunrise technique for treating this many, many years ago. And I mean, you have to take the whole back off and, oh my God, that was just pretty extensive surgery. And I think we can get pretty good results using this minimally invasive approach. So here we are protecting this scalp above the dura below and doing it with, and then getting cautery of the bone you send the suction electrocautery And wound closure is very simple. All it takes is just a couple of 4-0 monocryls. We inject a little bit of local right at the incision site, and that does, that is the surgery. So let me go on and finish this video presentation by showing you some of the results, if we can go to the next slide. So these surgeries are really, I mean, almost bloodless, but with very little blood, there's nothing really to worry about back there. So we've calculated about 15 cc and surgery takes about an hour. This shows the estimated blood loss with age, and really it's all over the place. It has nothing to do with age. So one of our older kids, we have very little blood loss. Surgical time, again about a little under an hour. Length of stay, very patient went home the next day. And not a single patient of our cohort series has needed a blood transfusion, either intra or post-operatively. So a couple of clinical cases to finish. Here's a little girl who was almost seven months old. Surgery lasted 60 minutes, 15 cc blood loss, went home next day. Again, classical synostosis. This is documented by CT scans, she came with a CT scan, but you can see that flattening here, not from positional molding, but from actual synostosis. And at a year later, you can see the correction. Now they see how her head still tilted a little bit in the back. That takes that takes time to fully correct. But even at a year, you can see a marked improvement in the back of her head, which is what we were concerned with. And we did that with just a couple incisions without needing to do a very extensive surgery. Here's his boy, five and a half months old, 43 minutes. Now this, this was a little more complicated of a more difficult case. You can see this child had a very large anterior fontanelle. And for some reason is some deviation of the face anteriorly and he had significant posterior plagiocephaly. And typical of lambdoid synostosis you can see there's significant down displacement of the involved ear and mastoid suture. Here's a kid at two months of age. A lot of these kids do have this notorious cephalic looking face. They don't quite a 100% normal. Even at five months of age is still looking pretty abnormal. Six months of age, still has that large forehead. Ten months of age. Fourteen months. But the back has improved significantly. I mean, he came in with his face. Where we didn't, we weren't treating that. But here's a kid at four years of age with the face looking significantly better. But if you look at this, this is pretty impressive, I thought, correction of the skull base. I mean, it took a long time, but you can see that this ears have pretty much realigned themselves. He still had a little bit of flattening here. You can see maybe perhaps in this suture of the scar on the nasal. However, when you look at the kid from above, you can see that over time, it has been significant improvement of the shape of the head. And none of the thing that didn't happen is his face kind of strained out over time at four years of age. So I think for an operation that really took less than an hour, no blood loss and an overnight stay, that's a pretty darn good result. I'll show you another one, five months of age and operation of an hour. Again, these kids do have a unique look to them. Here's your classical lambdoid synostosis that I showed you with all those changes. So this is to show this was not positional molding, but true lambdoid. Even at a year later, she still has a much better looking face than before. And she did correct her posterior deformity. A three and three month old child. Again, you can see this typical lambdoid synostosis, same problem. With pretty decent correction, even at a year, you could just barely see that one scar there. So you got before, at a year, and then. So even at a year already, we were seeing significant improvement of these kids. And I think the last patient I wanted to show you, this one again, you can see the classical lambdoid synostosis with the different changes in shape and as the child progresses over time so that now four years later, the kid looks relatively normal. So I think in summary, I do believe that we both believe that approaching lambdoid synostosis doing these minimally invasive surgeries is probably a much better option than taking out the entire back of the skull and putting patients through a significant risk by doing those big operations that we used to advocate a long time ago.

- Thank you so much, Dr. Jimenez for agreeing to share all of your accumulated wisdom with us today for, this the second of the two part series on craniosynostosis. And I'm sure I speak for all of the viewers of WNS Operative Grand Rounds, we're very grateful for you taking the time today.

- Thank you.

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