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 Doctor Jason Pomerantz. I'm a craniofacial plastic surgeon and co-director of the UCSF Craniofacial Center. Doctor Peter Sun and I are going to talk with you today about Diagnosing and managing abnormal head shape in pediatric patients. We have no relevant disclosures. The goal of this talk is to explain the basic elements of diagnosis and management of deformational and synostatic plagiocephaly and to understand the time course of diagnosis and treatment. Which are critically important for the outcomes that we all are looking for. Uh, we manage, uh, patients, uh, with abnormal head shape as a team, uh, endeavor at the USSF craniofacial Center, and this involves specialists from multiple areas in 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, uh, 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, metopic, and lambdoid sutures which are abnormal in synostatic plagiocephaly and normal in deformational plagiocephaly, which is critical to understand in in terms of understanding the treatment options. A key aspect of primary care for craniofacial patients is understanding the differentiation between deformational and synostatic plagiocephaly because. As you know, deformational platocephaly or molding is managed non-surgically with position modification or helmeting, and we generally follow the 2016 guidelines for management of patients with positional plagiocephaly 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 helmeting both in terms of the time used by the parents as well as the knowledge and expertise of the orthotist is critically important and we have a craniofacial. Orthotist 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. The head skull changes dramatically over the first year of life in terms of its malleability as the 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 helmeting 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 craniosynostosis, so this is synostatic plagiocephaly, 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 suture. The skull grows perpendicular to each suture, and so the deformities associated with suture fusion are directly related to that. If you see here in this image of a skull with sagittal suture craniosynostosis, the sagittal suture is fused and missing, and the skull is unable to grow in this direction. So the biparietal area is narrow and the head. Compensates by becoming too long in the anterior posterior direction and this is also shown schematically in this diagram. Here All of the different single suture synostoses behave concordantly. For example, coronal suture, if a. Here right coronal suture is fused. The skull cannot grow perpendicular to that suture results in a flat forehead 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 3 or 4 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 craniosynostosis versus deformational plagiocephaly in about 95% of cases without any imaging at all, ultrasound, which is a benign form of imaging, is being developed for detection of craniosynostosis but is 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 craniosynostosis 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 in 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 craniosynostosis and goals in treatment. The indications and goals of treatment of craniosynostosis 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 coronal and atopic synostosis. There are two main surgical options for treatment of craniosynostosis. To reiterate, surgery is the only treatment that can improve and correct craniosynostosis. The two treatment options are 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 generally a longer operation with greater blood loss and a larger scar. The other option is strip craniectomy where the synostotic 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 4 or in some cases up to 5 months of age, and we have moderately strong evidence at level 2 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 metopic craniosynostosis. 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 and involves decision making by both the surgeons and the parents. Doctor 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 craniofacial center. It's based in two campuses, one in Oakland, uh, one in San Francisco, and there's a comprehensive team of Medical specialists that works with us to provide optimal care for these patients who 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 cranial facial centers because not all centers have all these tools available to them either for the lack of technology or a particular uh philosophy. So I think, I think that's really, really, uh, 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 helmeting 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, uh, particularly the minimum invasive surgeries which require post-operative helmeting. And what's really nice for the families is that we are able to, uh, 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 orthodist 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. Doctor Pomerantz will uh illustrate some of our other techniques. It's very important, as we've talked about, for early referral because within the 1st 3 to 4 months of life as the skull is growing, the brain is growing rapidly, the head size is rapidly uh 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's shape that determines the skull shape. You know, if our brains are square, our skulls would be square, but our brain is round and if we can get to the patient within the 1st 3 to 4 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 a head, head, head shape that is normal and much natural appearing than actually the bigger surgery. So in terms of timing, we need to see these patients early on so we can get them to the OR by 3 to 4 months of age. And in terms of the scar, uh, it's obviously not a trivial thing. We can offer a much smaller scar and we can see the patients early on. And um do the strip craniectomy uh through the scar. Also, there's less transfusion, obviously, less anesthetic 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 to 4 days as opposed to 5 to 6 days. And I think we get terrific outcomes for both, um, but it really, a lot of it depends on when we see the patients. So, early referral, very important. This will be an example of a calvero vault remodeling where we do the bigger bigger operation. It utilizes a bicronial incision and then we shape the bone into a desired shape and we really can get a fantastic outcome because we're able to shape the bones uh the way uh that uh we want to. And this is a preoperative case and you can see postoperatively, we have a much rounder, much normal head shape. We do have the scar. 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, uh, parents often wonder why we zigzag it. This way, it's uh 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 a helmet afterwards. And the helmet is typically worn for 3 to 6 months. Um, sometimes we see the shape we want in 3 months. Sometimes patients need a longer period of helmeting. So this is a study we recently published that illustrates for the first time that a minimally invasive approach for metopic cranial synostosis can be better than the open approach. This really has not been shown before and this is falling in patients over a long period of time, um, which has not been done. So, um, I think it's really important. I think it, uh, changes the way we, we think about metopic cranial synostosis. I just wanna go over some of the data in this study. So, of course, metopic craniosynostosis had been treated with the open technique with good results. Um, the strip craniectomy, the minimally invasive approach was pioneered first by Doctor Jimenez, um, 20 years ago. And uh we've taken this to, um, the metopic. Craniosynostosis and really follow these patients um over time. Data that's in the literature, uh, it has quite short follow-up and it does not have a lot of the objective measurements that we've taken uh to uh 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 utilized the standard anthro uh um metric measurements, uh, um, and they were equivalent in terms of outcome. Um, patient satisfaction was also very similar. Um, but this is where, uh, it gets interesting cause 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 show the pictures, uh, 3D pictures as you see here with our 3D camera to a group of adolescents and it had them grade who looked more normal. Um, and also, we had the craniofacial 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, uh, we're able to achieve this outcome because over time, we've modified our, uh, surgical technique to incorporate just some nuanced changes. For example, we used to shave the corner, um, 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 it to shaving it from the inside and that's giving us a much better outcome. In conclusion, our study, uh, showed that, um, the strip craniectomy is associated with superior long-term appearance on really vigorous measurements. Um, That's not been utilized um in other studies before. And as it turns out, the scar is not a trivial uh matter for, for the patient's self-image. And it should be, this approach should be considered in all patients who we get to see uh in a timely fashion. So this is an example of the bichronal scar. Obviously, this patient has shorter hair, so it's seen, but you can see clearly the difference between a bichronal scar uh versus the minimally invasive scar, which is a 2 centimeter incision uh in a 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 Doctor Pomranz go on to uh discuss more uh the other surgical techniques and other uh classes of patients with uh cranial synostosis. For the majority of patients, Especially the ones with fusion of only one suture, the techniques uh we just described are applicable and important. But in more complex craniosynostosis 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 suture synostosis, and these have evolved over the last 10 to 15 years. For example, this baby has Munca syndrome caused by an FGF receptor 3 mutation, has fusion of multiple sutures, including both coronal sutures and the right lambdoid suture, which results in a very significantly abnormal and asymmetric head shape and also has 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. 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 sequela 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 4 months of age, at which point we use cranial distraction, cutting the bone and applying these devices through a coronal 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 distractor 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 we're able to obtain very nice outcomes, normal head shapes and appearances, and prevent sequelae of intracranial pressure increases in even complex patients. This is an example of the 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 craniosynostosis 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 craniosynostosis with a 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 neuro as the neurosurgeon, Doctor 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 we're able to obtain. A normal skull in terms of shape and volume, very reproducibly. This is a large operation and not our preferred approach. We prefer to treat patients early and for the reasons we discussed, we have these approaches available to treat any patient with synostosis. 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, uh, refer patients early. We're happy to see them. We can clinically diagnose the head shape without imaging in 90 to 95% of cases. We are happy at UCSF to see patients without imaging and without CT scans or X-rays. Position modification and helmet therapy are effective for deformational plagiocephaly without synostosis in sagittal and metopic cranial synostosis 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 4 months of age for coronal and multiple suture cranial synostosis treatment is still typically cranial vault reconstruction, which we do at about 1 year of age. And we use cranial vault distraction in complex patients who require expansion earlier. Please do not hesitate to reach out to Doctor Sun or myself at our email addresses here on this slide, and please visit our web page cranialfacial.UCSF.edu where you can learn more about our team and also directly refer patients. Thank you again.
