Hydrocephalus: Management of a Lifelong Problem – PediaCast 452

Show Notes 


  • Dr Jeffrey Leonard visits the PediaCast Studio as we consider hydrocephalus and explore the cause, symptoms, diagnosis and treatment of this lifelong problem. We hope you can join us!


  • Hydrocephalus




Announcer 1: This is PediaCast.


Announcer 1: Welcome to PediaCast, a pediatric podcast for parents. And now, direct from the campus of Nationwide Children's, here is your host, Dr. Mike.

Dr. Mike Patrick: Hello, everyone. And welcome once again to PediaCast. It is a pediatric podcast for moms and dads. This is Dr. Mike, coming to you from the campus of Nationwide Children's Hospital. We are in Columbus, Ohio. 

It's Episode 452 for February 12th, 2020. We're calling this one "Hydrocephalus: Management of a Lifelong Problem". I want to welcome all of you to the program. 


So this is becoming a habit in 2020 of covering medical conditions that are not the most common ones to impact children and teens, but they are not exactly rare either. So these are conditions that are out there, if not in your family, perhaps the family you know, or a family who attend your child's school or lives in your neighborhood or is a member of your faith community.

So it's good to have awareness of these conditions even if your child is not affected because you will, at a minimum, cross paths with families who are impacted by things like rumination syndrome, which we talked about in Episode 449, polycystic ovary syndrome we just covered in 451, and today, hydrocephalus.

We also want to raise awareness of these conditions because like most things in medicine, the sooner they are diagnosed and treated or managed, the better the outcome for kids and their families.

And you never know when one of these conditions will strike close to home. So it's good to have some working knowledge even of the less common pediatric medical conditions. Because when we're not aware, there can delay in diagnosis or inadequate treatment, and our children can suffer because of that.


So today, our attention will focus on hydrocephalus. You've probably of heard of this condition, hydrocephalus? But what exactly is it? Who is at risk for being affected? How does it happen? What signs and symptoms are associated with hydrocephalus? How is it diagnosed and treated? And what are the long-term consequences of this life-long problem?

We have answers for you this week. We're going to talk all about it. And to help me with those answers, I really do have a terrific guest visiting the studio, Dr. Jeffrey Leonard. He is Chief of Pediatric Neurosurgery here at Nationwide Children's Hospital, and he will be here shortly.

First though, I do want to make a quick public service announcement for both parents and also the pediatric medical providers who are the audience. 


As you know, antibiotics are fabulous, they treat bacteria illness, they can save lives. But we also want to be good stewards of antibiotics because when they are used inappropriately, antibiotics contribute to the development of resistant or difficult-to-treat infections. They can kill our normal or good bacteria, which we are discovering is associated with the host of additional potential problems.

They also expose our patients and our children to potential side effects and complications. So antibiotics are great when they are used wisely, not so great when they are used without thought or caution. And one of the ways that we can improve antibiotic stewardship is by using them for known bacterial infections. That means not using antibiotics to treat viral colds and coughs. That's one way we can make a difference. 

Another way is by reducing the duration of antibiotic treatment for certain illnesses. And one of those illnesses, in fact, it's an illness that affects lots and lots of children this time of year is the common ear infection. Or, as your medical provider calls it, acute otitis media, also known as a middle ear infection.


In the past, the recommended first-line antibiotic for pediatric ear infections was high dose amoxicillin, which is a penicillin antibiotic given for ten days. However, in 2013, the American Academy of Pediatrics released a new clinical practice guideline which continues to recommend high-dose amoxicillin as first line treatment, unless there is a penicillin allergy or recent use of amoxicillin in that particular child.

However, in an effort to encourage antibiotic stewardship and practice antibiotic stewardship and also exposed children to fewer days of antibiotic therapy, the new recommendations calls for five to seven days of treatment, rather than ten days of treatment for kids over the age of two years with non-severe ear infections. 

To be more specific, the new guidelines call for seven days of treatment for those who are two to five years of age and five to seven days of treatment for those over the age of five, again, with non-severe acute otitis media.


Now, you may be asking what is then severe ear infection? That is defined as having a fever a 102 degrees Fahrenheit or a severe pain associated with that particular ear infection. In other words, it's more than just a little ear discomfort or pressure or even mild to moderate pain. It's severe pain. And in that case, or when there is a high fever, the AAP says go ahead and use ten days of treatment regardless of age. 

And these recommendations by the way are based on scientific evidence and expert opinion. If you're interested in reading the guideline for yourself, I'll put a link to it in the show notes for this episode, 452, over at pediacast.org. It's a publication entitled The Diagnosis and Management of Acute Otitis Media.


So why are we making a big deal about a guideline that was put into place back in 2013? It's a good question. And the reason is that lots and lots of children over the age of two with non-severe ear infections continue to get ten days of amoxicillin for their ear infection. Why?

I think there's two reasons. Many doctors and other providers are not aware of the 2013 AAP guideline. Also, old habits are hard to break. So my goal here is to raise awareness and to try to get providers and parents to think intentionally about the length of treatment for children over the age of two years with non-severe ear infections. 

And yes, this is an important thing for parents to think about as well because there's nothing wrong with asking your doctor why he or she is prescribing ten days when the guidelines call for five to seven days. 


Now, your provider may have a perfectly good answer to that question. And I would absolutely encourage you to follow the advice of the provider who knows your family, examines your child and is aware of your child's unique circumstance. At the same time, it's good to think about why we do what we do, and to question and to advocate for our children based on evidence and expert opinion. 

Again, I'll have the AAP guideline for the treatment of ear infections in the show notes for this episode, 452, over at pediacast.org. 

Along with an excellent blog post on the subject which you can share with family and friends on social media or through email, just to help raise awareness, it's called Ear Infections: Why Kids May Not Need 10 Days of Antibiotics. 

And for the doctors and other medical providers in the crowd, would you please consider taking our very brief five-minute survey? In fact, I'll bet you, it will not take you five minutes regarding your preference for treating ear infections as we explore practice patterns and try to gauge the effectiveness of educational messages like this one. 


And I'll put a link to that anonymous survey in the show notes for this episode again, 452, at pediacast.org. You'll also find it by clicking the Survey tab at the top of any page over at pediacast.org. Again, survey, Length of Treatment for Ear Infections, you'll find it there at the website. 

All right, let's move on. I want to remind you that the information presented in every episode of PediaCast is for general educational purposes only. We do not diagnose medical conditions or formulate treatment plans for specific individuals. If you have a concern about your child's health, be sure to call your doctor and arrange a face-to-face interview and hands-on physical examination. 

Also, your use of this audio program is subject to the PediaCast Terms of Use Agreement which you can find at pediacast.org.

So, let's take a quick break. We'll get Dr. Jeffrey Leonard settled into the studio and then we will be back to talk about hydrocephalus. That's coming up right after this.



Dr. Mike Patrick: Dr. Jeffrey Leonard is chief of Pediatric Neurosurgery at Nationwide Children's Hospital and a professor of Neurological Surgery at the Ohio University College of Medicine. He's a principal investigator in the Center for Childhood Cancer and Blood Diseases at the Abigail Wexner Research Institute here in Nationwide Children's.

And he's published over 100 scientific papers in medical journals, has a passion for helping children and their families who are impacted by disorders of the brain and spinal cord including hydrocephalus. 

That's the topic of our podcast this week, so let's give a warm PediaCast welcome to Dr. Jeffrey Leonard. Thanks so much for stopping by and visiting us today. 

Dr. Jeffrey Leonard: Happy to.


Dr. Mike Patrick: So let's start with just a brief definition. One of those words I think a lot of families have heard of but there's still a lot of mysteries surrounding, what is hydrocephalus? How would you explain that?

Dr. Jeffrey Leonard: So the way I start explaining hydrocephalus when I see people in clinic is I just explain it as a system. Hydrocephalus is an abnormal collection of spinal fluid. So what most people don't realize is that spinal fluid is made on a day in-day out basis. It's made in the choroid plexus, circulates and is absorbed up in the middle of the brain in an area called arachnoid villi. 

So that's a system that is both produced and made, produced and made. And you or I, as adults, probably make somewhere in the 350 to 450 cc's per day range. 

So the reason I present it as a system, that if there is a blockage of that, that's hydrocephalus. Meaning, it can't get through to get absorbed in the back of the brain or the absorption doesn't work. Or in certain types of tumors, there's too much production where you make 6 to 700 cc's in a day, in which case, you're going to need some help with absorbing that.


So when there's that imbalance that the patient has hydrocephalus, and the manifestations of that are a little different depending on the age of the patient. If you or I were to get hydrocephalus, we'd get sick, we'd start throwing up, people would know something's different.

If children, little babies get hydrocephalus, their heads start growing because their sutures aren't fused. Sutures usually fuse somewhere between years one and two. And once that cranial vault becomes a rigid container, their head no longer gets bigger. And they all of a sudden start getting sick or manifesting the same signs that say adults do or younger children.

Dr. Mike Patrick: In the middle of the brain, there's ventricles. So these are just hollow cavities filled with spinal fluid?

Dr. Jeffrey Leonard: So the brain is made up of what we call two lateral ventricles, a third ventricle, and a fourth ventricle.


So what I tell people is that the lateral ventricles are C-shaped structures located in each hemisphere of the brain. And this is connected to the middle where the essentially central part of the brain which is the third ventricle, which drains into the fourth ventricle, which is the part that is the cerebellum or back of the brain. 

Dr. Mike Patrick: So the fluid starts in these ventricles, that's where it's made and then it flows out. And then, to the outside of the spinal cord and around the surface of the brain before it's reabsorbed. What's the function? What's that fluid there for?

Dr. Jeffrey Leonard: So one of the most common illustrations that I use is everybody's in high school or grade school done-drop-the-egg-from-the-wall school experiment. And how do you get the egg to survive? It survives by putting it in water.

And essentially, the CSF is the shock absorber for the brain. So what it does is it prevents anytime you get hit, anytime you move... Let's say for instance, you run into a door, or whatever accident may happen, it shields the brain from that kind of impact. 


There are kind of things that it does because there's all sorts of irons, calciums that go through that. So it's a transport system as well, but it's a shock absorber for the entire system.

Dr. Mike Patrick: So it really helps protect the brain and spinal cord and then nourishes it, too, with nutrients and glucose and takes waste products away and all those kinds of things.

And I love the way that you describe that as a closed system. So we're making spinal fluid and then it acts as a shock absorber, gives nutrition and then it gets reabsorbed as it's kind of used up. And anything then that increases production, decreases absorption, or causes a blockage, you're going to have a buildup of that fluid.

Dr. Jeffrey Leonard: And the differentiation is important then for pediatricians, for ER physicians. It's the very first thing I ask is what's the diagnosis?


And they say, "Well, hydrocephalus." And I say, "Well, so why does this child have hydrocephalus?" Because what that does is that it immediately keys me in to is this child going to get sick really quickly? Is this the child that may or may not need a shunt? Is this the child that has some other problem going on? 

The reason I say this, for instance, if a child has a shunt and is a myelo, those are the type of patients that their ventricles don't change. They can get sick very quickly, so those shunts need to be addressed in a very timely manner. 

Whereas, kids with pseudotumor, which is more of a problem with absorption or an imbalance in the production and absorption, what happens with those children, those children don't get sick very quickly. They present with very slowly progressing headaches, maybe vision loss. So there is zero emergency to treating those children. So it's very different depending on the diagnosis. 


Dr. Mike Patrick: And really, the how common that it is. And so, the epidemiology of hydrocephalus is also going to be different depending on what that underlying cause is. 

Dr. Jeffrey Leonard: Correct.

Dr. Mike Patrick: In terms of congenital hydrocephalus, so babies who have a problem right from birth, how common of an issue is this? 

It's the most common diagnosis where we interact with pediatricians and the public, and often pediatricians have several kids with hydrocephalus in their entire practice. Whereas, for instance, kids with brain tumors, oftentimes, you'll see many pediatricians have just one child in their entire practice that has a brain tumor.

This is important because at least for our neurosurgery practice and across the country, it varies between 30% to 50% of the cases that we do. So it's one of the most common things that we do. 

Dr. Mike Patrick: One of the numbers I had seen for infants with hydrocephalus is that it affects in the United States about one in every 1,500 births or so. So I mean, it's not common but it's not rare either. It's really one of those things that we all need to have some working knowledge of to identify when this issue occurs. 


In terms of increased risk, who is at more risk for developing hydrocephalus in infancy? What are some of the things that, if those are present, we got to really be thinking about the possibility of hydrocephalus.

Dr. Jeffrey Leonard: So the most, the biggest group right now that we have to think about hydrocephalus on is the premature infant. Oftentimes, we are very good at keeping children alive at 23, 24 weeks. And that's great but it also requires a lot of work and access to medical care to make that happen. 

Because one of the things that happen when you get the really premature infants is that they have hemorrhage. And there's a grade of hemorrhages. There's four grades, with the third and fourth grade causing hemorrhage into the ventricle and causing accumulation of spinal fluid, via an imbalance in the system. 


This occurs because the absorptive system doesn't take kindly to having blood products in there. It becomes fibrous and it doesn't work as well. 

Dr. Mike Patrick: It clogs the filters, so to speak. 

Dr. Jeffrey Leonard: Exactly. It's like having a furnace filter that you need to change. And, unfortunately, you can't change it. So you need to help it, so you need to figure out some way to bypass. And the way these children manifest is they come in the NICU or when their heads get bigger and bigger and bigger. 

And the common term that we see is that we ask is the children's head circumference, or the other term's OFC, crossing growth curves? So if it's crossing growth curves, that mean there's an imbalance in the system and we need to step in to address it because in that age group, that's when all the connections are made, that's when the brain's still developing. 

Brain development occurs far after these children are born. So we need to do whatever it takes not interfere with that or to allow it to progress in the way it's going to. 


Dr. Mike Patrick: And some of those risk factors, if we think about prematurity, then the risk factors of prematurity also sort of then become risk factors. So things like low socioeconomic status, poor prenatal care, all the things that sort of lead to prematurity, we're going to see in those families.

Dr. Jeffrey Leonard: Absolutely correct. 

Dr. Mike Patrick: We'll see increased hydrocephalus there. So that's hydrocephalus associated with birth and congenital hydrocephalus, but you can get acquired hydrocephalus as well, which we've talked about in terms of like pseudotumor which we've talked about in this program before. Also, just tumors in the central nervous system that may be compressing or causing blockages externally.

Dr. Jeffrey Leonard: So what my research interest is and everything else is brain tumors. And that's sort of what my initial part of my career was made, and it's being able to resect and diagnose brain tumors. And, oftentimes, in kids, the most common place where children present with brain tumors is in the back of the head or the posterior fossa. 


And they can, since it's such an uncommon... And brain tumors are 3,500 to 4,000 in the United States per year, is people don't expect that. And what happens is they manifest because they progressively get bigger and bigger and bigger and block the spinal fluid. And oftentimes, they present with headaches.

And by headaches to be specific, these are the kids that go with headaches where they have headaches every single morning. They get up, they throw up, and once they get up, their drainage improves and they go about their day. And it happens every single morning and it goes worse in crescendos. 

And unless the clinicians or the parents are advocating for it or the clinician is thinking about it, you need to get a scan of the head to make sure nothing is going on. 

Dr. Mike Patrick: And as those ventricles get full of that blocked-up cerebral spinal fluid, then those exert pressure on other parts of the brain and that's what ends up causing the symptoms that we see. 


Dr. Jeffrey Leonard: Yeah. What they cause if they first cause those symptoms, and late causes, they cause what we call herniation, which that compartment is only so big. Because by that age, the sutures have all fused and it can't get any bigger. The head reaches adult size by age five. So it's not going to grow much beyond that.

So when you get an extra mass in there or extra spinal fluid, something's got to somewhere. So what happens is the brain ends up herniating against the brain stem and causes you decreased consciousness. It causes cranial nerve findings. And the children come in very sick if they waited that long.

Dr. Mike Patrick: So a take-home for parents and for medical providers, when you hear of these symptoms, waking kids up in the middle of the night or they wake up in the morning, and they have headache, nausea, vomiting, maybe difficulty concentrating, confusion, but that improves as they're up and about during the day and that happens morning after morning, "Hey, this is a significant thing."

Dr. Jeffrey Leonard: I think the way I still talk about it, because a variety of... Where I talk about headaches, I don't want everybody to run around and be paranoid, "I got headache and it's brain tumor."


But some of the larger studies, ER studies, they take 100 headaches, only 9 to 10 of them are going to have intracranial abnormalities. So it should not be the first thing you jump to. But if you go two weeks and they don't respond to conventional therapies, yes, you need to look and see. So I usually wait two weeks to see what they look like and then you do an intracranial imaging. That's a perfectly reasonable way to approach it.

Dr. Mike Patrick: Great. Before we move on to diagnosis and treatment, you mentioned myelos and we have a lot of parents out there who are listening. So what is myelomeningocele and spina bifida and how does all of this relate to hydrocephalus?

Dr. Jeffrey Leonard: So spina bifida is an abnormal development of the back or abdominal development of the nervous system. They present either in utero or at birth if they haven't had prenatal care. And they find that their legs don't work as well and their nervous system hasn't completely closed over. 


The reason you talk about hydrocephalus in terms of myelomeningocele is oftentimes they're associated. About 60% to 70%  of people with myelomeningocele have associated hydrocephalus. And this is important because you need to look at that after you've end up closing the myelo. 

And closing the myelo occurs within the first 24 to 48 hours after the child is born or prenatally if they go to a center that has prenatal closure for this. It's becoming increasingly more and more common.

Dr. Mike Patrick: As we think about the symptoms that we mentioned with increased pressure in the head, what are some of the other things? So, you know, you mentioned a small number of people with headaches are going to end up having brain tumors and hydrocephalus. What are some of the other things we should be thinking about in terms of bad headaches and things?

Dr. Jeffrey Leonard: Well, there's headaches. There's children, they sometimes complain of double vision. So when they sometimes complain of double vision. One of the most subtle instances of hydrocephalus that we see is what we call is a sixth nerve palsy. 


Now, what the sixth nerve does is it causes your eye to move laterally. And this is the longest nerve in the entire central nervous system that's very sensitive there.

And sometimes, I've seen kids present with either shunt problems or hydrocephalus with an isolated sixth nerve palsy. And you can just see it. You ask them, "Hey, look to the right," and they can't. 

So there's a variety of other things that can very subtle. It's the normal nausea, vomiting, hydrocephalus. When it gets really late, they have trouble looking up, what we call sundowning. Oftentimes, I've seen several children with this where the really young children, where they have trouble with their eyes looking up. 

So when you look at them, look at them in the eyes, what you're seeing is you're seeing their eyeballs. You can't see their eyeballs because they're buried down looking towards the ground and they can't look up. 

Dr. Mike Patrick: What about migraine headaches? How do we differentiate those? Because sometimes there can be neurological symptoms and nausea and vomiting with migraines.


Dr. Jeffrey Leonard: That's why those are absolutely the great pretenders. And people, when you have teenage kids that have both migraine, headaches, and hydrocephalus, those are very difficult to sort out.

What I usually do is there's some common risk factors that you ask these kids. You go, "All right, does light and loud noises make it worse? Do you have a family history of migraines?" What happens when you give him Imetrex or a migraine cocktail? Does that help? If it's a shunt problem, that won't help it. If it's a migraine headache, then potentially we'll start to see some things. 

We have a variety of questions we also ask. So it's not just one simple question, it's an entire pattern. Like for instance, if somebody comes in and has migraine headaches and a shunt, but it's been six years since they actually had a shunt revision, then we're going to go with migraine headaches all the way, unless they show problems. So I ask them when's the last time they had the last shunt revision. 

So most of our shunt problems happened within that first year. Most of our shunt infections happen within that first six months. So if it's been four years since they've actually have a shunt revision, that's not going to be the first thing we jump to. But again, as you correctly point out, there's a whole lot of overlap between those symptoms.


Dr. Mike Patrick: And this is I think one of the reasons why you really need a good provider who you trust, who's going to take the time to kind of tease out what symptoms are going on and look at all the data points and come up with the decision better than maybe a computer could. 

Dr. Jeffrey Leonard: Well, yeah. This is what I keep telling our families. It's developing a relationship with your provider, your neurosurgeon that knows you, that knows when you walk through the door, rather than have a meeting with somebody for the first time. 

Because there's a relationship that occurs as you see them on year in and year out basis that is important in deciding, "Do I image this child? Does this child look sick?" No, he probably has migraine headaches, because he's presented the last three months with this. Rather than going to an ER provider that is seeing them for the first time. 


Dr. Mike Patrick: Yeah, who's more likely to jump to doing imaging because they don't really feel comfortable knowing this family and kind of what you've gone through in the past. 

Dr. Jeffrey Leonard: Right.

Dr. Mike Patrick: So especially  for that patient who has not been diagnosed with hydrocephalus, how do you go about making that diagnosis and differentiating it from other things?

Dr. Jeffrey Leonard: Well, there are several things that we use. And again, to emphasize it's not one particular question, it's an overall pattern that you see. We look for imaging studies. So when the ventricles are enlarged and it's done in an acute fashion, you see what we call transpandemal flow. That's an imaging finding that says that's acute hydrocephalus. The pressure of the ventricle is forcing spinal fluid out of it. 

We look for the folds of the brain or what we call sulci and gyri and are those pushed or squished because they're being compressed by the spinal fluid. 

We look for head circumference for the really young kids. Is it crossing growth curves? Basically, for the older children, how are they doing in school? Are we seeing changes that are different from it was six months ago? What I generally try to do is a complete pattern. 


The ones that we all said, nausea, vomiting, decreased mental, those are easy. But it's when things happen that are not as easy or more subtle or more insidious in the onset, then it becomes a bit of a problem. 

Those head shapes, when I walk in the room, if somebody has a big what we call frontal bossing, yes, the child's going to get imaging before they leave this campus because that can be a manifestation of elevated intercranial pressure.

Dr. Mike Patrick: So once you make that diagnosis, how do you then go about treating hydrocephalus?

Dr. Jeffrey Leonard: Well, we have a variety of tools available to us. Probably the best way to do that, start with a really young children. We generally can put shunts, and which are the mainstay of what we do, in kids 2 kilograms or greater. So we have some temporary devices called ventricular access devices that we put in say the NICU. And the only reason for that is to allow them to get older. 

So we just access that on a daily basis because the spinal fluids made then, they can't drain it. So we just need to help them. 


So once they get to be 2 kilos, we talk about putting a shunt. Now, a shunt is essentially just a diversion technique for the spinal fluid, what we take from the top of the brain down to put it wherever we need to. 

So the options for putting it are in the belly. Everybody likes to say, "Well, do you put it in the stomach?" It's like, no, it's the covering around the stomach. It's the covering around your intestines, it's called the peritoneum. And what the peritoneum is it's an epithelial lined cavity that provides an absorptive surface of the brain. The absorptive surface in the brain is not working, so we need to find another one. 

Dr. Mike Patrick: So you're just diverting the fluid through a tube from the brain down to the abdominal cavity. 

Dr. Jeffrey Leonard: I'm just a fancy plumber.


Dr. Mike Patrick: And the tube is just right under the skin, correct?

Dr. Jeffrey Leonard: Correct, it's right underneath the skin. So other areas we can do that, we can put it right into the internal jugular vein. And we deliver right back to the blood system so that it can restart the whole circulation.


The other thing that we can put, and people don't realize this, is we can sometimes put it in the pleura if we're struggling because that is another epithelial lined cavity that we can use for absorptive purposes. 

So the point is we have a variety of options available to us that can provide absorption. So it allows us to deal with the CSF that is not being absorbed normally in the brain. 

Dr. Mike Patrick: And so the tube, the end of the tube that's in the head is on the outside of the brain or inside the ventricle?

Dr. Jeffrey Leonard: That depends on what we're draining. In most of these situations, the ventricular system is what needs to be drained so we'll put it the ventricle. So we plan for that. So everybody wants to know, how do you choose where to put it? Well, we usually put it in what we call the right frontal. 

The right frontal is an area where sort of my wife likes to think that guys use about 10% of the brain. Well, this is 90% that we don't use. 



Dr. Mike Patrick: So let's say for instance, well, the best plan for that is we plan for the worse. So let's say by putting it in, we have a hemorrhage or we cause injury in some way, the chance of affecting speech, the chance of affecting movement, that's not there. So we can get away with that, so that's why we put it here or here. Next choice is going to be here or here. 

So we know in the brain where the safest places to put it and then we tunnel it down behind the ear, making it as unobtrusive as possible for this children. So they can go back being in normal life. 

Dr. Mike Patrick: I would imagine you have to have some way to control the amount of flow that you're draining off because you don't want drain off too much of the fluid. So how do you control that?

Dr. Jeffrey Leonard: That's exactly correct. The next system is both the tubes. The next system that's important is the valve. The valve is a pressure controlled device that works by just opening at a certain pressure and closing at a certain pressure. So what it does is it opens and drains a bunch of spinal fluid, until the brain reaches a pressure where the valve closes. 


And what happens is that this control, this is sort of best approximation. The sad part about hydrocephalus is this is the way we've been treating it since the 1960s. And the new development associated with that is what we call a siphon device. 

So the really young kids, that doesn't matter. But as we get older and we start standing up like you or I, the teenagers when they grow a bit, they can siphon. And by siphon, I mean, you know like when you try to drink gas out of your dad's car, you do the tube, put it in and put it down. So gravity helps.

Same thing happens with hydrocephalus, there's a pressure differential between here, between the head and the abdomen. And what happens as they get older, the pressure differential increases and they just drain more. So we now have devices that control rate of flow called anti-siphon devices. So that prevents kids from, which is a very difficult thing to treat is what we call ventricular syndrome.  

And what happens is those kids is I ask them for postural, their headaches get worse when they stand up and worse when they lay down. So that's another component of what I question the children about when they come in complaining of headaches.


Dr. Mike Patrick: And the adjustments on the pressure opening and closing and the rate of flow, those can all be changed, even though this is a tube that's underneath the skin?

Dr. Jeffrey Leonard: Correct. We often change them by magnets. And we don't change them often because no one's ever been able to show, it's like having five shunt valves in one. And what we do is when I change it, I tell families it's going to take two or three days for you to feel normal, to readjust what I've just done.  So you cannot come in and just want it on a day in-day out basis and say, "I've got a little headache and can I change it one click to the right and one click to the left?" Because you'll be in and out of here all day long. 

There are some kids that are very sensitive to a particular pressure. There are others that I use to control the drainage when they have very severe forms of hydrocephalus. And we want to control the way in which the head is actually is drained so we don't get cranial deformities. 


Dr. Mike Patrick: Are there situations where you don't have to use a shunt but instead, you can actually directly open an obstruction that can be in the system?

Dr. Jeffrey Leonard: What people don't realize is how fast... That's one of the reasons I went into neurosurgery. This is a field that is changing very very rapidly. So even when I was in training, this particular procedure called a third ventriculostomy wasn't used. 

It was used very rarely. And we started using that in my training because the camera's gotten good enough. And the reason, the most important thing about the third ventriculostomy is what we call obstructive hydrocephalus. We use that when there's an obstruction that's blocking the flow of the spinal fluid. 

And we do this by making a hole in the bottom of the third ventricle, then the CSF can get out and be absorbed in a normal fashion. And it's one of the most common places that we see and we do  it in kids that have aqueductal stenosis. And by aqueduct, I mean the blockage between the third ventricle and fourth, and in kids that have tumors that occur in the posterior fossa.


So it's a common thing that we see and depending on his diagnosis can be effective over 80% of the time and prevent children from actually needing a shunt. So what's also happened is that we've also developed another technique. So what we used to do, and it started in Uganda because over there, you don't have shunts, right? You have to go out and pay for your own shunt.

And I imagine, you can't come to Nationwide and tell how to get your shunt, "You have to go out to Rosauers to buy a shunt to bring it to the hospital." That's what was happening there. And most of the folks couldn't afford it. 

So what they figured is, well, let's look at the CSF. Eighty percent of our CSF is made in the choroid plexus. What happens if we begin co-angulating it? And what borne out of that is the third ventriculostomy plus choroid plexus cauterization. Showing you could avoid a shunt in the newborns over 70% of the time if they have things like aqueduct stenosis and even in the children that have evidence of prematurity and hemorrhage as well. 

Because you don't have to deal with all of these spinal fluid at that time. Less than half is what's required to be drained, because I actually presented a paper that calculated the drainage per shunts. We just completed what we call a shunt check trial that showed that we don't have to drain all of the spinal fluid within the shunts. 


Dr. Mike Patrick: It must be some job to explain to families, like these are the choices and this is why we should do it a certain way and kind of have that sort of shared decision making to really get families on board when you're going to operate on the kid's brain, right?

Dr. Jeffrey Leonard: Yeah, they do. And it varies, too. But at the end of the day, what they'd end up going with is they say, "If this is your child, what would you do?" And I say, "Well, I try to avoid the shunt if you can, because it's kind of like the yellow brick road. Once you're on it, it's very hard to get off." Because shunts are lifetime events and very often, we're unable to get them off.


And we're able to avoid it, it's sort of a completely different ballgame for all these children. And a shunt by itself doesn't mean you can't play football. Then, they all said, "I can't go to school." I have kids that are now 20-plus years old. We have engineers. 

So I know with hydrocephalus, if you don't treat, the treatment of hydrocephalus, the idea here is not to cause a trouble by the treatment.

Dr. Mike Patrick: In addition to the neurosurgical treatment for these kids, it really takes the whole team of other professionals helping. Who's on your team?

Dr. Jeffrey Leonard: So it's the whole aspect of, you know, this is a day in and day out, this a chronic problem that must be cared for in the course of the child's lifetime. So we have nurse practitioners that we've all trained in how to do this because what we tried to do is we have clinics all days of the week.


So if they need to come in for it to be evaluated to see, so they know the people. They can come in and be seen by somebody with experience, that has been trained by me or our attendings rather than coming to an emergency room and meeting somebody for the first time. 

Neuropsychologists, oftentimes, these kids have trouble in school. So we have, oftentimes, they're going to neuropsychologist to end up with educational help as they move through the school system. So they're provided the resources they need to progress and develop. Because ultimately, we want to turn them away from Nationwide and have them leading normal lives. If I'd done that, I succeeded. 

Dr. Mike Patrick: Is there an increase in epilepsy seizures in kids who have these problems?

Dr. Jeffrey Leonard: No, no. Only about 7, it's not a big part of that. If we have to end up revising them and they have infections and that's a different story. But in the ones what we like to do is only about... Shunt malfunctions only present with seizures about 7% to 8% of the time. And that depends on who you read. So there's some pluses and minuses out there.


What we see in this situation is so the biggest things that everybody completely agrees on is that infections are bad. So we do everything we can to keep infections... Our infection rate is here 2% or less depending on the year. 

So that's important because we will cause seizures, we will cause loss of IQ. That is well established around the field. 

And the seizures, to answer that, that's dependent upon do you have other developmental abnormalities. There's a whole lot of factors that go into what happens there. 

Dr. Mike Patrick: And if that is occurring, then the neurology folks may be involved. Then, if there is other hits to the brain, then physical therapy or occupational therapy, speech pathology and all those folks may also be involved.

Dr. Jeffrey Leonard: Those all help with the care of these children. So we have a wide range of kids. So it's seizure kids developing with tumors that have seizures and hydrocephalus so it comes in all flavors.


Dr. Mike Patrick: And what is the long-term outlook then? You mentioned this is a life-long problem. And so do they graduate from the pediatric neurosurgeon to the adult neurosurgeon? Or do you continue seeing them for their whole life or is that still something that's... Who manages these kids as they grow up? 

Dr. Jeffrey Leonard: There is a person that is devoted a lot of their career goal at OSU but oftentimes we  don't end up transitioning them because what we see... Here's the best way to answer that, within the first year after you put a shunt in, about 30% of them require revision. Another 10% the next year, 10%  the year after. 

So about 50% of the kids are long-term, they don't need any, or the shunt's going for a long period of time. So what we like to do is we just come back and see them on year in-year out basis.


And then, as they get older, the incidence of hydrocephalus and shunt malfunctions in adults, it's rare and rare as you can really spread out when you need to see them. 

But this is where the adult medicine, adult neurosurgery, they haven't really done very well in dealing with chronic disease. So the myelos end up staying here to be dealt with, kids with hydrocephalus end up staying here to be dealt with. Because we have developed that, we understand the history. We have all the imaging studies. And it's much easier for us to make the decisions on what to do with them.

Dr. Mike Patrick: And I would imagine from a family standpoint too, it's really hard to change once you have established trust with someone who's really taking care of your child right from the get-go.

Dr. Jeffrey Leonard: And that's one when Ed, my predecessor was very good at that and took care of a large number of large number of hydrocephalus kids in young adults in the community doing it that same way.

Dr. Mike Patrick: Tell us about the other things happening in the Neurosurgery Program here at Nationwide Children's. 

Dr. Jeffrey Leonard: Well, quite a bit and we're now up to five people. And we have every single aspect of neurosurgery covered. We have a very strong epilepsy program. We are doing things like deep brain stimulation, laser ablation, all the very complicated hemispherectomy procedures for them. 

We have a robust movement disorders. We have one of the biggest spasticity programs in the country, where we deal with kids with CP. The premature infants often have cerebral palsy. And that's also a chronic problem for this children. And if they have it, next question is how do you deal with it?

And what you have to do is there is a variety of ways to reduce tone. And dorsal rhizotomy is one of those. So it's a procedure that wasn't being done before I came and we've now managed to ramp up. And that again, like anything within these kids, it takes a team, whether it be the physical therapists, the PM&R folks, the neurologists, all to help care for and get the best outcome for this children.


We have craniostenosis for head shape and then we have brain tumors as well. So we have a reach across... What we've done is we now do, of the 800 and some odd cases that we do ever year, over 200 are coming from out of the state. 

Dr. Mike Patrick: The epilepsy surgery, I find that really fascinating. How do you do that?

Dr. Jeffrey Leonard: Well, it depends on the types. So the important thing to emphasize is that starts with a very in-depth evaluation beforehand by our pediatric neurology colleagues. They do advance imaging. They bring them in to monitor them on a day in-day out basis and then they look at their clinical situation.

And what you try to do is you try to figure out are the seizures coming from a particular area of the brain? You look clinically, you look electrographically, and you look with imagery. And if they are, we try to monitor that. And by monitoring that as we stick multiple electrodes in the brain and then monitor these children over the course of anywhere of one to two weeks, depending on how many seizures they have.


Dr. Mike Patrick: And these are going to kids who medicine is not working for their seizures. 

Dr. Jeffrey Leonard: So that's a very good point. These are medically refractory. And what happens is that it means they failed two medicines. And once you start failing those medicines, the chances of being seizure free just dramatically drop. And what happens is that they start progressing. 

After that, they go on to being evaluated by the Epilepsy Surgery Service and then they move to conference where they get that what we were talking about earlier, shared decision making and decide what the best treatment would be for these kids, whether it be to oblate the hippocampus, to take out an entire hemisphere if the child has both doesn't move one side of the body and has seizures that are coming from one side that cannot be controlled. 

Or to take out the temporal lobe, oftentimes, in adults, they have seizures that just come from the temporal lobe. So a temporal lobectomy could potentially, in 60 to 80% of these kids, make them seizure free. So those are the kinds of things we're looking for.


Dr. Mike Patrick: And that's one of the sort of unique things I think about neurosurgery is that almost no cases are exactly the same, right? Just each one is so unique and you have to think about creatively what's the thing that's going to help these patients in the end the best. And it's not always the same from one kid to another.

Dr. Jeffrey Leonard: And what people also don't realize, it's a field that's changing so fast. A number of these procedures like deep brain stimulation, this is another alternative for epilepsy where you put leads into the hypothalamus and stimulate them to interrupt the circuit that is causing epilepsy. This procedure, when I was training wasn't done.

So these are things that your surgeon, your program needs to continue to learn and adopt or create if they're getting on the leading edge of all these treatments because this field is moving very fast. And this is why I went into it because it's an exciting field and it's going to make a whole lot more progress in the next 10 to 20 years. 

Dr. Mike Patrick: Yeah, it will be exciting to watch. 


We really appreciate you taking time out of your busy schedule to stop by and talk to us. We'll have a link to the Pediatric Neurosurgery Program here at Nationwide Children's Hospital in the show notes for this episode, 452, over at pediacast.org. 

We also have from our Nationwide Children's Health Library some things that you may find interesting to read or to share, more on hydrocephalus, also on spina bifida and myelomeningocele. We'll put those again in the show notes. 

But once again, Dr. Jeffrey Leonard, Chief of Pediatric Neurosurgery here at Nationwide Children's Hospital, thanks so much for stopping by today. 

Dr. Jeffrey Leonard: Thank you very much.



Dr. Mike Patrick: We are back with just enough time to say thanks once again to all of you for taking time out of your day and making PediaCast a part of it. Really do appreciate that. 

Also, thanks to our guest, Dr. Jeffrey Leonard, chief of Pediatric Neurosurgery here at Nationwide Children's Hospital. 

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Thanks again for stopping by. And until next time, this is Dr. Mike saying stay safe, stay healthy, and stay involved with your kids. So long, everybody.


Announcer 1: This program is a production of Nationwide Children's. Thanks for listening. We'll see you next time on PediaCast.

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