Babies’ heads can look odd for a variety of reasons, some benign and some requiring prompt, expert care. Winson Ho, MD, describes the head conditions he commonly sees as referrals; explains how to evaluate macrocephaly and other issues; and describes current practices when treatment is needed, including the importance of timing. Bonus: View a remarkable video of endoscopic ventriculostomy for hydrocephalus.
Great. So, yes. So I'm gonna just talk a little bit about some of the most common referrals that I uh got from, get from the community. Um As mentioned, you know, I'm Winston Home with the uh new faculty here at U CS F actually. Um And we're gonna talk about, we're gonna talk about macrocephaly play and, and some of the more common conditions we treat in pediatric neuro surgery. Um Just that as an introduction, I'm one of four pediatric neuro surgery faculty, um Doctor uh Curtis Dr Nolan Gupta, Doctor Peter Sun and my partner. Uh We all four of us cover both the San Francisco um campus as well as the Oakland campus. Um Two of us are based mostly in San Francisco and two of us are based mostly in Oakland. Um And as mentioned, I also have a research, you know, effort. I have a N I H funded laboratory studying uh immunogen, you know, immunotherapy for adult and pediatric brain tumors. Uh So that's one of the uh reason I actually came here. Um just as a quick background, um did my education and at Yale for both my undergrad and MD uh did my residency at the joint U VA N I H program, followed by pediatric fellowship at Salt Lake City. And then I've been in faculty at uh U T. Austin, one of the uh new medical school actually, um um one of the first faculty who joined the department uh for about three years before I came here. Um you know, in terms of the cases that I treat, this is a summary of the cases I did, you know, during that three years. Um you know, of course, as a pediatric neurosurgeon, the most common thing we deal with the CS F diversion V pe TV S. I'm also doing a fair number of epilepsy followed by everything else that um sort of we deal with in the pediatric neurosurgical um uh uh practice. So, uh as mentioned, I'm gonna talk to you about frequent referrals, uh things that I see most often in in my clinic here and the for first and foremost, is big heads macrocephaly. And um and uh oftentimes uh it is a typical uh of a growth curve like this when um you know, the head circumference are jumping percentile. So, um so this is sort of a really um I think a good framework to think about macrocephaly. Um Most of the time as we all know, uh macrocephaly is benign. Um But usually we get concerned when you see that crossing percentile curve on the conference chart. Um And so I think it's very reasonable depending on the age of the patient, obviously, um to get an ultrasound if the fin still open or get a rapid MRI. Um and if the scan is normal and there's no signs of, of increased intra pressure, uh I think it's totally reasonable to follow. Now, of course, if there are signs of increasing pressure, um referring to me, uh or two, it's, it's absolutely, um, I think the right thing to do or if you know there's concern, um You know, we could also be the ones who order those imaging as well. Uh But usually I would, the first thing I would do is get a head ultrasound or wrap an MRI. So distinguish between macrocephaly and hydrocephalus obviously is very important because one is a benign condition and one is a, is a condition that require treatment. And the nice thing about infants is that unlike in kids, uh unlike in adults where if you have hydrocephalus oftentimes it's sort of an emergency because you have increased acute, increased in uh in pressure, however, in infants, because you have an open and font now, almost never. Is it an emergency where um even with somebody who had pretty significant hydrocephalus. Um and the reason is because open font and allow the um uh pressure to be alleviated like a sort of safety valve and also the skull is pretty malleable. So, uh it would manifest as an increase in, in head circumference rather than in, you know, acute increase in venal pressure. Um, there are certainly times when, um, you know, we have to do things a little bit more urgently. It's pretty rare but it's usually in the setting of, you know, a persistent apnoea and cardia, for example, or if, you know, there's such a bad vomiting that you have dehydration or really severe down gaze palsy, those are signs that we would be more worried that there's sort of imminent, you know, um you know, herniation and things like that, but it's pretty rare. Uh But what are the physical signs and clinical signs that you look for between a benign microcephaly versus hydrocephalus? And the most important is a fel examination. Obviously, you know, it's like a window to the pressure of the brain. Um You know, if, if hydrocephalus often is much more tense, usually, the rule of thumb is that if the fel is pooching up above the level of the skull, um uh in a uh non pulsating manner and that's something that you'd be worried about. Um uh you know, on imaging finding, you know, hydrocephaly is often uh more associated with interest size. Uh Whereas uh benign microcephaly is often associated with extra axial food collection, which I'm gonna show you a little bit about um other clinical signs including descended scalp veins um in hydrocephalus, um splayed sutures. Uh Again, the rule of thumb is that you run your finger over the sutures and you can fit your finger in there. Um That usually suggests that the sutures are splayed. Um And of course, the treatment uh is very different. Now, this is obviously concerning if you get an imaging like this, if you have that sort of this big. Um and that we would be concerned about hydrocephalus. Um The so the most common cause of hydrocephalus that we tend to see um is actually posthemorrhagic hydrocephalus. Um you know, as we know what that are associated with premium I V H. Um So kids that are uh pre uh premature has high, higher uh tend to uh increased risk of having intra hemorrhage. And that intra hemorrhage is associated as posthemorrhagic hydrocephalus. Um The other uh rather common things that we sometimes could see is a congenital reason of hydrocephalus is aqueductal sonos. As you can see here in this image where the, you know, this is the third ventricle, this is fourth ventricle and this is the aqueduct can kind of see a little veil uh right over the aqueduct that's blocking the normal flow of spinal fluid from the third ventricle to the fourth ventricle and, and to the spine. Um Other um causes common causes includes posttraumatic postinfectious spina bifida obviously is one. Um and then other things, other obstruction like tumor, uh post fossil tumor or dandy Walker syndrome. The to treat hydrocephalus is a critical one that's faced by pediatric neuron, frequently, sorry um, it's a cute adult setting. I'm sorry. Hold on one second. Um, ok. And so when do we, so even if it is hydrocephalous, we oftentimes don't immediately, um, commit ourselves to treatment. Um, because, um, because as mentioned, you know, there's AAA malleability of the, of the skull. And so, um, even when I see big vents, unless, you know, there's, you know, clearly signs of increased hydrocephalus, we don't necessarily have to immediately uh uh um perform CS F diversion. Um because, you know, doing a or that version is, it's often it's a lifelong commitment, right? And so there are definitely uh instances where you have what's called a where the ventricles are large, but then the patron do not have signs of increased hydrocephalus. In those cases, I would actually watch and wait. Now, what are the treatment of hydrocephalus? And I think, I think we've all probably know the most common tried and true treatment strategy is uh ventricle partial shun has been done since the sixties. Um And it's really just, you know, uh you know, established a bypass uh of the CS F from the brain to the belly to partial space. Uh The part is, is the most common one, but of course, it could be atrial or um a um or a plural if for whatever reason, the, the partial space is not available or is hostile. Um Now, the other option that um we actually really want to consider when it's possible is uh what's called an endoscopic third ventri colostomy. Um And the idea is that um uh if there is some kind of obstruction like, you know, aqueducts that I've shown you, uh we could actually go and create a space at the floor, the third ventricle to bypass any obstruction. So, um as you would imagine, uh it works better for certain types of, of hydrocephalus such as obstructive hydrocephalus. If you have a tumor here, if you have the ecto glioma, for example, which oftentimes is block off the aqueductal space. Um This would be a great option because you just create an bypass. Um that it could be the CS F that are produced up here can then drain into the pre pontine system that could ultimately get absorbed into the, into um uh into the uh Venus uh outflow. Um uh However, um that's not, you know, 100%. There's certainly communicating hydrocephalus that could be treated uh with E TV. S um that have a little lower success rate, but it still sometimes works. It's, you know, it goes to show that we don't completely understand the ideolog the path uh mechanism of um you know, hydrocephalus. Um Here's a, just a quick video, a quick picture and video later on of what E TV is like. Uh This is the endoscope that's placed in uh the lateral ventricle and you're looking at the frame and roll here. This is the sep uh the septum and this is the qut plexus. Um And, you know, first we, you know, put the camera into the lateral ventricle, we'll be looking at the four M M and roll and then be through the four and Monroe would be the uh the third ventricle as you can see here. Uh We would then advance the camera a little bit further into the third ventricle and then, uh you know, identify the sort of anatomical structure with the mammal bodies, the infant recess, and then there's little veil that represent the floor of the third ventricle. And then we uh generally, then, you know, insert an instrument and basically just poke a hole into the fourth on the, on the floor of the third ventricle. Um The only thing that we sometimes most worry about anatomically is that the hole needs to be made right in front of the basil artery was kind of uh you know, socked in between the mammary bodies. So, anatomically, there are certain uh people who are, you know, very hard to do the E TV, because there's not a very near space between uh basal artery and the space that we could create. So we often times get a MRI or even sometimes AC G who identified that basal artery because um you know, while the risk is low, um and it hasn't happened to me personally, but it's certainly reported that you could, you know, perforate a basal artery and that could oftentimes be a fatal complication. Um This is a sort of video of uh so what we, you know, do during the, the case, you know, we already made a hole on the third of the ventricle. We can see how the, the third, you know, the floor, third ventricle sort of pulsate uh along the flows of the pulse, pulse and CS F. And we put a little full uh uh a, uh a uh um, a balloon through the hole that we created and try to expand that a little bit further. And then sometimes we could use an instrument to do that as you can see here. Um And then as we, um, make the hole a little bit more bigger, we can actually then, um, put the camera in a little bit more closely and then you would actually see a very beautiful anatomy where you can see the basal artery and sometimes you could actually see some pre poin scarring. Uh That oftentimes is, you know, a negative predictor of how likely the E TV is going to work or not. Um Here you can see that we're repeating and putting in a balloon and trying to, you know, expand that a little bit more. Um, you know, with the idea that you don't want, you know, the, the tissue to be flopping around too much that it could scar back. Um Because that would be the primary reason why the E TV could fail. Um And you can see now here, we would then usually advance scope a little bit further. So we can actually take a peek uh at, you know, the anatomy and making sure that, you know, the membranes is all dissected off, the liquid membrane is dissected off. But that's a basil artie right there. That when you make the hole initially, you will try to avoid, uh you know, injury that because that would be um you know, devastating. So when does E TV works, you know, obviously E TV is appealing for various reasons, right? Number one, it doesn't um uh it doesn't require any foreign body. So it's not something that um you know, have, you know, high risk of infection and need for um uh potential vision in the future. Um And so, um however, it doesn't work that well in all circumstance. Um most importantly, the age of the patient is a big pre predictor of whether the E TV, success uh is successful or not. So this is called E TV. Success, success, success score score. And the three main uh variable is the age ideology and whether they had previous shunting or not. Um And each point uh after you add about adds up to the percentage of success uh over, you know, in, in, in two years after the E TV. So as you can see here, there's a big jump in success rate. Uh once you get past one year of age. Um And then we know that, you know, as, as mentioned, the ideology is important. So if you have a clearly obstructive ideology like aosis or tumor that has, you know, a high rate of success, um and then, um and then whether you have a history of previous stunting or not uh alters that success a little bit, not that much, but by about 10%. Um And so, um oftentimes when I see infants who um who uh need hydro, uh who need CS F diversion, I would offer, you know, V P shun uh when they're really young because E TV doesn't work that well in those situation. Uh And then as they get older, it's always possible to go back and uh consider doing E TV. Um because um uh uh because at that point, uh you can't, the, the E TV, success is higher and so um you could potentially render them shunt free. Uh You take a little bit of hit in terms of having a previous shunt but not that much, but about 10%. So, um so that's Hydrocephalus, we talked about that. Um But what about the benign condition, the benign condition of uh of macrocephaly? Um It's often associated with this um Enlar Sub Duo Collection. Um And there's various names that you probably have heard. I call it Bestie Benign Lar space. Other people call it, you know, be nice. Suburu, Benign Hydro. Um and it's oftentimes congenital, um one of the most common, the first thing I would often ask when I see someone Macey is, is there are family members who have big heads. Um Because oftentimes that's uh something that, that you, that you have that family history. Um And this is very characteristic, you, you know, see this jump in the percentile and then it falls back to the and then it goes up to a very high percentile and then it follows a growth curve and settle in a very high percentile. And so this is what the imaging often looks like. Um So instead of the being bay is a sub space that's and large. And um however, it is important to distinguish uh benign, you know, a or sub from a subur which we sometimes see with non accidental uh trauma. Um In this case, um uh you could have increased in inter uh increased pressure from a chronic subdural. In which case, sometimes we have to put in either temporary drain or even a subdural shunt. And the way you tell the difference is that because the cortical draining vein is in the sub space, um if you have an enlarged sub space, you can clearly see the the draining veins in that, in that enlarged space. Whereas if uh if you have a sub, which is uh more one layer superficial to the sub um layer, um you have blood that's pushing the sub, the the uh the draining vein inside into the brain. You can see here you don't see any veins or, you know, you see flow voids in the, in the MRI that's, you know, suggest those are the veins and here all the veins are pushed down against the surface of the brain. And so this is a picture of a chronic subur all compared to in a benign and space and almost always, um you know, be is something that you could continue to observe. Um very, very, very, very rarely do I have to treat. And I don't think I personally treated one uh who have, you know, truly best. Um But, you know, as I said, chronic subdural is a different it, uh it's a different um condition that sometimes need treatment. And so the other frequent rever referral is a big uh is the odd head shapes. Um And um and so I'm just go over some of the most common, um you know, ran and Asos uh that we uh see and uh what are the treatment option for those? Um As we all know, um our skull comprises of various different sutures. The sutures allows the skull to expand as a skull matures and, and large uh to accommodate the growth of the brain. Um So you have your metopic sutures, your corn sutures, your sale, sutures, your Lambo sutures, each of these and, and squamosa. Um each of these sutures kind of have a different timing of uh fusion uh in a normal situation. Meat topic usually close pretty quickly within the first couple of months of, of life and then close and when you're in your third, um So if you have premature closure of the sutures, then your skull is unable to expand in that um uh orthogonal direction that the sutures are allowing you to expand as a result. You have this abnormal head shape that are very characteristic of uh of one or if multiple sutures were are f so just go over quickly. Um uh you know, each of those um the topics and sources um usually present fairly early on, as I said, because it closes, you know, uh you know, the first few months of life. And so when they prematurely close, it's usually when they're either fusing in, you know, in uh uh fet or soon after birth. And because, um and the most characteristic head shape that it gives you is trigly. And so you have this very, you know, characteristic, you know, triangle like head shape. Um um It's also not uncommon that we see this uh um metopic ridges that we see, which is, uh which we sometimes see when the suit, when this uh when the metopic close, just a little bit uh earlier than usual, you see a very tiny uh uh or sometimes pal ridge that doesn't look like that, but it looks at a little bit sort of odd um and in those cases, most of the time, they kind of even out over time and we rarely have to operate on those. And, and so generally, I would have a little bit higher threshold to really operate on topics and those sources and that there's a pretty significant tri and then saal um synostosis is the most common. Um This is the most common synostosis that we deal with. Um is when the sage suture closes prematurely, the sa sutures allow you to expand your biparietal dimension when this fuses prematurely, that cannot happen and your skull kind of compensate and instead elongate um this way. And so it's very characteristic. You see that so long he shape. Uh The other characteristics include, you know, pretty significant frontal bossing or ox what called ox bullet. Um Some people have more prominent uh frontal bossing. People think that that's because, you know, the part of the, the structure is what fuse first and that's what can get you the frontal bos or if you're further back, then you have a very prominent bullet. So really pointy occipet. Um um but so this is very, very characteristic. And then the second, more uh most common is after saul is a Ronal systo. Um and oftentimes more commonly as a unilateral premature fusion. Um And in that, in that case, is this the side where it is fused, you can't expand, you can't expand your frontal, uh your frontal bone. So that the contralateral frontal bone become more prominent. And you also have this very characteristic sort of, you know, harlequin deformity where your, your oral rim is kind of malformed compared to the other side. On the other hand, if you have, you know, fusion of both coronal sutures, then you have this sort of breaky because both your frontal uh bone cannot expand. And so you kind of compensate by having this really tall, you know, head shape. And um and, and so, you know, when you have, um uh when you have more than one future, that fuses oftentimes, we worry about what's called a syndromic uh uh synesis. Uh where in addition to just having isolated, you know, uh a fusion of the uh of the suture, you have other um features associated with that as well. Uh And in those cases, it's more likely that you would have increased inal pressure because um you have more than one suture that are fused and then the least common is the lambdoid synostosis. Um It is important because, you know, it's just important to distinguish this from the way more, much more common thing, which is the position of uh clay when you have the lambdoid sutures being fused. Um your um what you see is that your ear. So on this side, which is the flat side of your head, which is the side where the suture is fuse your ear actually. Um um uh it's uh push poster, whereas you have contra frontal bossing. Um So you could almost have this trapezoid kind of head shape. Um And this is important because, you know, we also have flat head. And as I said, you know, positional aleph is a more common thing, you know, we see um in which case, um the head, the ear is actually pushed forward and you have uh uh you have ipsilateral frontal bossing rather than contra frontal bossing. And I'll have a picture of that to illustrate that in a bit. Um And as I mentioned, when you have um multiple pen uh sutures that fuse, oftentimes you worry about whether they're syndromic and uh the syndromes are the ones that um have other associated abnormality and the more common of the three uh syndromes are the Cruz A and the Phifer. Um And as you probably know, um these are associated with uh um a lot of times, you know, cranial facial amorality such as, you know, a hyper hyper or me face hyperplasia and cruz on uh for a, you have the of, of the limbs and, and for fiver you sometimes get syn syn syn, you also have this sort of peat nose and, and sort of bit toes. Um And as um and as mentioned, um you know, one of the things that we uh often talk about in treating uh CSIS is whether, you know, there are two reasons to treat them, right? 11 is, you know, the form we had. Um the, so the psychosocial aspect of, you know, development, um that's number one, the number two is whether um there is increase in pressure, uh when you only have a single sutras sporadic cases, um the rate of increased pressure is probably not very high. Um The studies that were done, you know, you know, years ago when they actually put an IC P monitor to actually measure, you know, in, in pressure in kids with um suggest probably 15 20% of the time you actually have borderline or high IC C P. But when you actually have um uh multiple Rios that or in the setting of syndromic crises, that rate is much higher. So obviously, if you have increased crinal pressure or signs of interest pressure, then we have to treat that. Uh But I think in this country, you know, anybody who has even a sporadic C is would, would be offered treatment. Now, uh this is one thing that I would actually is very important in terms of timing of referral because um the treatment for cryos is different if we get them early. Um because the skull at the point at that time is less mature and we actually have more mentally invasive option to treat CSIS when they're between 3 to 66 months and when they're older, beyond six months. Um the traditional method of doing a queen of vault reconstruction would be the sort of the only option and I'll just quickly show a couple of pictures. Um, so when they're really young, um, at least over the last 10, 15 years, um, uh, we tried to identify more mentally invasive way to treat this, uh, these conditions and now there are really two major, um, methods in, in addressing this. Um, it's, you know, in, in, in both of those is we would first do a strip creamy to remove the few sutures. And then, um, in one option is to put in what's called springs. Um, so they are basically, you know, just like springs that you, um, that you can actually place to continually expand, um, the, the, uh, the, the skull after, uh, it's a temporary implant. So we usually in, you know, place them here, for example, uh, between 3 to 6 months of age and then we'll follow them up over several months and to see that the spring would continue to expand, uh, over several months and most of the time, um, once the expansion is complete, your skull would almost, you know, have a prenormal shape at the time and then we go back and remove the springs. Now, the other possibility is do a strip cran toy and then, uh, do helmet, um, just doing stripy have been shown to not work. Unfortunately, uh, you do need help to sort of mold the shape into the shape that you want to correct that scho in this case with the, uh in, in this case with the services. Um So in those cases, you would just take off the bone and then you put the patient in a helmet over several months. Um Every center do it a little bit differently. I've seen centers do it for, you know, 2 to 3 months and I've, you know, also seen places where to do it 8 to 9 months. Uh But obviously, you have to be placed on a helmet for most uh hours of the day. Um And you also need to have a good or uh um uh orthotic company to help you potentially to modify the helmet. Uh Depending on how uh how the uh outcome is, uh how the progression of the head shape is. Um So when I was in Austin in Texas, um, you know, I've been doing mostly springs, but in training, I've done a lot of this helmet, uh both works pretty well. Most of the papers that are looking at it, you know, demonstrate they have pretty equal effectiveness in, in terms of uh adequate correction of the deformity, at least for Sagi. And so these are, so these are some of my, you know, cases and examples, you know, this is a very typical, you know, you know, so you can see here, um, the a suture is, is fused and have this really elongated head. Um So what we did is you can see here you know, do a strip cranny and then we put in three, sometimes four springs to basically yank this, um, this apart so that you can increase the biparietal dimension out their skull. Um And, you know, uh and as I said, we generally put them in around 3 to 4 months of age and usually by, you know, 6 to 9 months, the expansion is completed. And then we go back as a very small procedure to, you know, take the, take the, take the, um, the strands out. Now, uh, you know, obviously the, the downside of doing the sprints is that you basically commit yourself to two procedures. You have to go back and take them out. Uh, the upside is that you're gonna have to wear a helmet all the time, uh, with helmet, uh, you're only doing one, uh, procedure, but then you have to be on a helmet for a few months. Uh This is an example, uh, of a single, a single coral cress. Uh We put the two, uh, the two springs in. Um, and, uh, and I think this patient did quite well. We don't have to go back and do a cranial vault reconstruction, um, for that, and this is a helmet that you, uh, would have to place if you're gonna do the, um, helmet. Um, and then, uh, and, and if patients present, you know, older, um, older than six months, then, uh, we generally because the skull of the time is already much more matured and the um mall of the skull is much less than when they're younger. Uh The springs or the helmet would not work. Um And so this is the, a typical uh queen of vault reconstruction, um, where we basically remodel the, the, the, the skull by making bone cuts. Um And, you know, and, and plate them together and they are very various ways to do this. Almost every center has some, their little flavor of what they think makes it better. But oftentimes we just, um you know, uh you know, most of the data suggests that, you know, those procedures are pretty comparable in efficacy. And then, um and then, and then how to um as I mentioned, to distinguish the quo from the position of plagiocephaly, which is a much more common phenomenon that we see, especially after the back to sleep um uh uh campaign that to show that um sleeping in the back is, is, is better. Um It's often associated with Toto collis and it's most commonly, you can just treat that with repositioning and tummy time. Um And as I mentioned, the one important thing um to distinguish the positional plagiocephaly is with uh Laos and it's a really easy way um to distinguish it. Um because you remember um the, the previous picture where the um flat side um uh of, of the skull have your um um as your ear is being pushed poster uh by the flat, you know, by the side that's um uh fused. But in this case, uh on the flat side, the ear is pushed forward. And so is your frontal bone and so your, your frontal bossing is on the same side of your flat, flat side of the head. And so, uh that's sort of how you um distinguish um uh uh positional plato from lambdoid Cristos. Now again, lambis, the the most rare type of single sutra. So it's way more likely and much more common to have positional plato. And how about treatment for successfully, as mentioned, conservative management is the most common. You can do repositioning techniques if the patient have torto callus, um you know, physical therapy. Now, if um if the improvement uh does not, uh if the hedge tape doesn't improve over several months and by age 6 to 9 months, there are still pretty significant for bossing or er asymmetry. Uh then helmet is also an option. Um uh You know, the downside to that is that a, a lot sometimes you sorry. Um Sometimes the, the insurance doesn't cover it and it may require some adjustment and also it needs to be worn, you know, as often as possible, you know, save by more than 23 hours a day. Um uh But it, it, but it is a benign condition and it's mostly for cosmesis that you do that for. So, that's all I have today. And I'd be happy to answer any questions. Hopefully that's helpful and, um, uh, be of some use. Um.
