Emphasizing the value of early referral, specialists with the UCSF Craniofacial Center present essential information for pediatricians noting or suspecting plagiocephaly – including how to know what's actually abnormal when examining a baby's skull. The surgeons explain why imaging is often unnecessary for diagnosis; discuss how they manage both simple issues (positional head flattening) and craniosynostosis (fused skull sutures); and describe surgical approaches, including a less invasive technique that can optimize aesthetic outcomes, supporting the child's psychosocial well-being.
Hello. My name is Dr Jason Pomerantz. I'm a craniofacial plastic surgeon and co director of the UCSF Craniofacial center Dr Peter Son and I are going to talk with you today about diagnosing and managing abnormal head shape and pediatric patients. We have no relevant disclosures. The goal of this talk is to explain the basic elements of diagnosis and management of defamation allow and systematic play joseph and to understand the time course of diagnosis and treatment which are critically important for the outcomes that we all are looking for. We manage patients with abnormal head shape as a team endeavor at the USsF craniofacial center and this involves specialists from multiple areas and pediatrics. Today we're representing plastic and neurosurgery but we have individuals on our team representing genetics, pediatrics and many other specialties, all of whom contribute to care of these patients. Assessing skull shape can be difficult for individuals who are not used to it. And one relatively simple way to think about it is by understanding the major sutures that are involved in creating head shape. These are the coronal sagittal, the topic and lambda sutures which are abnormal in Cina, static play, joseph and normal in defamation, which is critical to understand in terms of understanding the treatment options. A key aspect of primary care for craniofacial patients is understanding the differentiation between defamation all and sign a static place because As you know defamation played awfully or molding is managed non surgically with position modification or helmet ng. And we generally follow the 2016 guidelines for management of patients with positional play Josepha Lee. Published by the Congress of neurological surgeons and endorsed also by the American Academy of pediatrics. We have all the facilities for training parents for position modification, for example, in the diagram on the left and also have the facilities for doing helmet therapy for remodeling the shape of the head, both at our Oakland sites and at our Mission Bay sites. We do have observed over time that the quality of helmet ng, both in terms of the time used by the parents as well as the knowledge and expertise of the orthodontist is critically important. And we have a craniofacial orthotics specialist at our Oakland site who exclusively manages patients with head shape differences. The main point to make in terms of referring patients with head shape abnormalities. And we will refer back to this point several times during this talk is the importance of early referral. Uh the head uh skull changes dramatically over the first year of life in terms of its malleability as a brain rapidly grows. And as the bone rapidly becomes more and more firm and hard. So in order to have effective head shape changes with helmet ng or positioning requires very early referral. As soon as there's a question that a patient may need diagnostic input or management input from the craniofacial team ideally within a few weeks after birth, in patients who have cranial anastomosis. So this is seen a static place. Sucessfully meaning one or more sutures are abnormal and fused abnormally. The shape of the skull and the intracranial volume cannot be corrected by positioning or helmet use alone. The different head shapes can be observed and diagnosed if thinking about the way the bone grows relative to the future, The skull grows perpendicular to each future, and so the deformities associated with future fusion are directly related to that. If you see here in this image of a skull with anastomosis, the future is fused and missing and the skull is unable to grow in this direction. So the by parietal area is narrow and the head compensates by becoming too long in the Antero posterior direction. And this is also shown schematically in this diagram here all of the different single suture behave concordant lee, for example, coronal suture. If here right, corona suture is fused. The skull cannot grow perpendicular to that future, results in a flat, far ahead on that side and compensatory changes on the other side. These can be diagnosed clinically in the majority of cases and early referral to craniofacial center. Makes available several different treatment modalities that become less available in advanced ages beyond three or four months. So when thinking what should I do next? For a patient with abnormal head shape, measurements of head shape are useful but we usually rely on our clinical assessment of the appearance and then decide on imaging. We at UCSF are happy and often prefer to see patients prior to imaging Because we're able to diagnose Kronos anastomosis versus defamation play, Josepha Lee in about 95% of cases without any imaging at all. Ultrasound, which is a benign form of imaging, is being developed for detection of Kronos anastomosis but has not yet tried and true. So when in doubt refer and we're happy to see patients as early as possible. There are important disparities in cronos anastomosis care, including ones that affect our patients in the Bay area, northern California and beyond. We've studied this at our craniofacial center at UCSF and have a publication and press in the Journal of Craniofacial Surgery. The important takeaway message from this study is that disparities can affect referral time to our center and the time of surgery and this impacts the types of operations We're able to offer our patients and the importance of early referral is stressed here when patients are referred early. We are able to offer the full spectrum of treatment options that we will discuss shortly. We have found that individuals of non white races and with Medicaid insurance are referred to the craniofacial center later and undergo surgery later. And this impacts the treatment options they have. There are several indications for treatment of Kronos anastomosis and goals in treatment the indications and goals of treatment of Kronos anastomosis are several. They include prevention of neurodevelopmental problems. Those related to increased intracranial pressure and brain growth and also normalization of head shape, appearance and craniofacial growth. This can also affect the eye sockets and globe protection, which is another indication for treatment especially for corona lemon topic Asus. There are two main surgical options for treatment of Kronos anastomosis. To reiterate surgery is the only treatment that can improve and correct Kronos anastomosis. The two treatment options or cranial vault remodeling or open surgery where the surgeons remove the bone, reshape it and replace it. This results in all of the work being done during the time of the operation but it's generated longer operation with greater blood loss and a larger scar. The other option is strip craniectomy where the sign a static bone only is removed and then helmet orthotics are used to allow the brain to drive the head shape to normal over the course of months. This operation is only an option in younger patients less than four or in some cases up to five months of age and we have moderately strong evidence at level two and now improved evidence from our center that this is a very effective and perhaps preferred treatment in the majority of patients with both sagittal and the topic cranial anastomosis. Both of these treatment options are good and effective and we offer both at the UCSF craniofacial center in certain situations, one is clearly preferable over the other and in some the decision is more nuanced than involves decision making by both the surgeons and the parents Dr. Sun will discuss. Now the different treatment options in more detail and preference for one versus the other. I just want to start off by saying that I think we have a truly special cranial facial center. It's based on two campuses, one in Oakland uh one in san Francisco and there is a comprehensive team of medical specialists that work with us to provide optimal care for these patients. We can often be very complicated and I think what is really special for us is on the surgical side, we offered a full gamut. The entire spectrum of surgical care from springs to vault expansion to distraction. And of course, I think what we're really good at is a minimally invasive approach. So with these tools we can tailor to specific patients in order to get the best outcome. And I think that sets us apart from other craniofacial centers because not all centers have all these tools available to them, either for the lack of technology or a particular philosophy. So I think I think that's really, really special and really important for patient outcomes. In terms of what I also think is a very special aspect of our center is that the orthotics, the helmet NG is done in house and on site. So on the Oakland side, we have an in house orthotic Person who has been doing helmets with us for the past 10 years. So with that expertise and by working together on site we can achieve the best outcomes following, particularly the minimum invasive surgeries, which require postoperative helmet me and what's really nice for the families is that we are able to adjust the helmet on site. So if we see you the patient and there's a little tweaking that needs to be done a little padding, a little shave off on the helmet that will give rise to the most optimal cosmetic outcome. We're able to have the orthotics do it right then and there while the family wait and they don't have to do another appointment. So I think that's really a fantastic feature. So talking more about the different techniques, I think it's uh important to just talk about strip craniectomy versus vault remodeling right now, Dr Pomeranz will illustrate some of our other techniques. It's very important as we've talked about for early referral because within the first three or four months of life as the skull is growing, the brain is growing rapidly, the head size is rapidly expanding. We can take advantage of the brain's growth to push out and achieve the nice round head shape. It's ultimately the brain shape at departments of skull shape. You know, if our brains are square or schools will be square, but our brains around and if we can get to the patient within the first three or four months of life and do just the strip craniectomy by removing the few sutures and then using the brain along with the helmet to achieve head shape. That is normal and much natural appearing then actually the biggest surgery. So in terms of timing, we need to see these patients early on so we can get him to the O. R. By 3 to 4 months of age. And in terms of the scar it's obviously not a trivial thing. We can offer a much smaller scar if you can see the patients early on and do the strip craniectomy through the scar. Also there's less transfusion, obviously less than aesthetic time. It's a smaller operation. And in terms of the postoperative care, they're usually in the hospital. In terms of the strip craniectomy for 3-4 days as opposed 5-6 days. And I think we get terrific outcomes for both. But it really a lot of it depends on when we see the patients. So early referral. Very important. This would be an example of a Calvero vault remodeling where we do the big bigger operation utilizes a by criminal incision and then we shape the bone into the desire shape and really can get a fantastic outcome because we're able to shape the bones. Uh the way uh that we want to and this is a preoperative case and you can see post operatively we have a much rounder, much normal head shape. We do have this car. This is an earlier picture of healing. It doesn't really blend in very well. We zigzag it actually because we don't want the hairline just to fall on the scar. So parents often wonder why we zigzag it this way. It's much better hidden actually within the hairline. So this slide shows a strip craniectomy or minimally invasive or often called endoscopic approach whereby uh two small incisions are made and then we go in between and just remove the fused bone. And then as we talked about we put them in the helmet afterwards and the helmet is typically worn for 3 to 6 months. Um Sometimes we see the shape we want in three months. Sometimes patients need a longer period of helmet. Ng So this is a study we recently published that illustrates for the first time that a minimally invasive approach for me, topic cranial sinister assis can be better than the open approach. This really has not been shown before and this is following patients over a long period of time um which has not been done so um I think it's really important. I think it changes the way we think about me topic cranial sinise. Oh sis I just want to go over some of the data in this study. So of course me topic radios and associates have been treated with an open technique with good results. Um The strip craniectomy and a minimally invasive approach was pioneered first by dr Jimenez um 20 years ago and we've taken this to um the topic Crane arsonist Asus and really follow these patients over time data that's in the literature uh has quite short follow up and it does not have a lot of the objective measurements that we've taken to study the outcome. I rapidly adopted the minimally invasive technique as soon as it came out. So we have a large experience with this um technique and we're able to study our patients. So we looked at longer term outcomes between the two approaches. As you can see they both yield very good outcomes as you can see on A. And B. But we really took a very extensive analysis of the deformity outcome. So you'll see that we've usual. Ized the standard anthropomorphic uh metric measurements uh and they were equivalent in terms of outcome. Um patient satisfaction was all So very similar. Um but this is where it gets interesting because we incorporated additional measures including psychosocial outcomes and and up to 15% of the open patients actually felt that their scars was an issue. In addition this has not been done before which is we showed the pictures three D. Pictures as you see here with our three D. Camera to a group of adolescents and that had them great who looked more normal. Um And also we had the cranial facial surgeons independently look at these pictures to see who had a better outcome. And in these analysis the strip or minimally invasive group clearly came out better. Also were able to achieve this outcome because over time we've modified our surgical technique to incorporate just some nuanced changes. For example, we used to shave the corner from the outside of the bone that actually created sometimes a little bit of hyper hypertrophy of the bone or overgrowth so we can see a hint of um lumps and bumps. Um So now we changed to shaving it from the inside and that's giving us a much better outcome. In conclusion, our study showed that um the strip craniectomy is associated with superior long term appearance on really vigorous measurements. Um that's not been utilized in other studies before. And as it turns out, the scar is not a trivial a matter for for the patients self image. And it should be. This approach should be considered in all patients who we get to see uh in the timing fashion. So this is an example of the bike ronel scar. Obviously this patient has shorter hair so it's seen but you can see clearly the difference between a by criminal scar versus the minimally invasive scar, which is a two centimeter incision in the V. Shape or that hugs the hairline. So these are some of our patients over time, they really have terrific outcomes. At this point, I'm gonna let dr Pomeranz go on to uh discuss more uh the other surgical techniques and other classes of patients with cranial sinister Asus. For the majority of patients, especially the ones with fusion of only one suture. The techniques we just described are applicable and important. But in more complex carino synced Asus cases, patients who have more than one suture fused, often caused by mutations in the FGF receptor pathway. The minimally invasive approaches are not adequate and in fact, we have evolved treatment paradigms for patients with multiple sutures, anastomosis And these have evolved over the last 10 to 15 years. For example, this baby has monkey syndrome caused by a F G F receptor. Three mutation has fusion of multiple sutures, including both coronal sutures and the right lambda suture, which results in a very significantly abnormal and asymmetric head shape and also as a much higher likelihood of developing increased intracranial pressure because the head shape is very asymmetric and multiple processes are occurring at once causing the abnormal head shape. The strip craniectomy and helmet therapy is not an option and we are also working against the clock in that earlier treatment is still important to prevent quality of intracranial pressure increase. Uh The treatment paradigm we have evolved for this and other centers have as well uh including University of pennsylvania where some of this was developed involves a staged approach, including treatment of the volume first at about four months of age, at which point we use cranial distraction, cutting the bone and applying these devices through a corona incision and then gradually expanding the bone expanding the skull a couple of millimeters per day. You could see the difference between these foot plates here and here, which results in a very significant increase in intracranial volume, makes room for the brain and decreases the forces that are causing abnormal head shape in the front of the skull, which gives the baby time for the brain to develop. Bone forms very aggressively and robustly in the space between the distracter devices, which is a property of infants and does not occur after the age of a few years in most cases and then this can be followed by treatment of the front of the skull, the so called frontal orbital advancement and forehead remodeling, which is done by a separate open procedure through the same incision. And by this stage approach were able to obtain very nice outcomes, normal head shapes and appearances and prevents the quality of intracranial pressure increases in even complex patients. This is an example of a spectrum of options available to us and even in patients who present late Either because they were referred late or could not access care. Interestingly with COVID-19 pandemic, we have seen an increase in patients being referred late to our centers. We have a much more difficult problem for the reasons we described earlier, here's an example of a sagittal crest anastomosis with with a very abnormal head shape. So the head shape gets worse with time in untreated cases and the skull is much thicker operating on it is more difficult and is associated with more bleeding and longer operative times. In those cases we have to have developed use of virtual surgical planning where we use the preoperative cT scan design the operation before and after. And we were able to use normative data of normal skull shapes and sizes to very closely match normal skull shape and volume for age before the operation even starts. So this eliminates much of the guesswork and thinking during the operation. We use templates and models to reconstruct the skull in real time. So as as the neurosurgeon dR sun is removing parts of the skull, we are fixating it separately in this recipient template and here's an example of a reconstructed skull matching our preoperative plan. Even in very difficult patients. For example, this patient has a VP shunt and has multiple few sutures, resulting in lack of bone formation in several areas because of increased pressure or able to obtain a normal skull in terms of shape and volume, very reproducible. E this is a large operation and not our preferred approach. We prefer to treat patients early for the reasons we discussed. We have these approaches available to treat any patient with acidosis in summary In diagnosing and managing abnormal head shape. Early diagnosis is really critical treatment ideally occurs in the first few months of life and if referred early allows for treatment options and optimal outcomes. So please refer patients early. We're happy to see them. We can clinically diagnosed to head shape without imaging and 90-95% of cases. We are happy at UCSF to see patients without imaging and without ct scans or X rays, position modification, helmet therapy are effective for defamation successfully without anastomosis in saddle and the topic. Since so sis in particular strip craniectomy and helmet therapy, we've shown results in as good and often better outcomes if we can treat the patients early, ideally under four months of age for corona and multiple future Kronos anastomosis treatment is still typically cranial vault reconstruction, which we do at about one year of age. And we use cranial vault distraction in complex patients who require expansion earlier. Please do not hesitate to reach out to dr sun or myself at our email addresses here on this slide and please visit our web page craniofacial dot UCSF dot e d u. Where you can learn more about our team and also directly refer patients. Thank you again
