Our next speaker is Dr. Bekele from Denmark on Cerebellar Mutism in Adults. Does it exist? Good afternoon. I'd like to thank the organizers of this year's WNS meeting for inviting me to give this talk. Well, let me start by apologizing to all of the people that are trying to find out whether cerebellar mutism are causing adults. I also would like to know myself. However, like Tony, I guess because I'm a replacement speaker, I'm actually not from Denmark. I'm from... Sorry. So, rather than speak on that, which I don't have any experience on, I'm going to share with you some of my experiences with the use of endoscopic thoracic ventriculostomy when combined with choroid plexus cauterization in childhood hydrocephalus. I'm from Lagos, Nigeria. I have no disclosures. Well, hydrocephalus remains very common, and it's probably the most common non-traumatic condition that we have to deal with as neurosurgeons, especially those that are pediatric neurosurgeons. With an estimated one in 1,000 live births, there are estimates as high as 200,000 new cases of hydrocephalus occurring yearly in Africa. And while shunts have traditionally been the main state of treatment, shunts are expensive. It's a multi-billion dollar industry in the United States. And one of my painful memories as a resident was having patients' children wait on the wards while their parents scrounged around trying to get money to procure shunts. Fortunately, we now have lower cost shunts, and through the work of organizations like IF, and so that scenario is less common. However, if you're not careful, you may be replacing one problem with another. Shunt infection rates are notoriously high, 5 to 8% even in the best of places. And in low- and middle-income countries, it's as high as 17%. And some workers have shown that you have as high as 40% shunt failure rate within the first two years. Okay. All right. And so sometimes a solution can become a problem. I don't know what your experience has been, but we see this sometimes in our setting. And the lesson, this is a child that's having a shunt protruding from the anal passage, and this is a child that has multiple shunts, and one of them has been disconnected. And obviously, this is shunt infection. So with that background, obviously, there's a great desire for viable alternatives. My endoscopic thought ventricles to me and coriolis catheterizations are by themselves not new. Over the last 5 to 6 decades, various workers have shown and demonstrated their efficacy in various settings. But in our environment, it's a more recent development. And literature is quite supportive of the use of ETVs in adults and in older children. However, its use in younger children and in infants remains a bit somewhat controversial, depending on who you listen to. However, when we look at the literature, most of the children or infants treated were treated with endoscopic thorn ventriculostomy alone. But more recently, addition of CPC has shown to improve the yield, success yield, obviously. Our understanding of CSF physiology and the pathophysiology of hydrocephalus is obviously still evolving. Over the last 100 years, the bulk flow model has been that which was propounded by Dandy talking about cerebrospinal fluid produced from the croeplexes and going from the ventricular system to the subarachnoid space to be absorbed by the arachnoid granulations in the duralvenous sinuses. Hydrocephalus, therefore, was thought to develop when there's an obstruction of the flow in the pathway, except in rare cases of croeplexes papilloma or carcinoma. Hence, the common use of the phrase obstructive or noncommunicating and communicating hydrocephalus. More recently, the hydrodynamic model has been developed to describe hydrocephalus developing from disorder of intracranial pulsations. The normal arterial pressure waves, when they enter into the brain, are dissipated through the subarachnoid spaces, the venous capacitance vessels, and also in the croeplexes pulsations. And when these pulsation absorbers are dysfunctional, the intraventricular pressure rises, and this is thought to lead to ventricular enlargement. Well, the question for the physician or the clinician is that what then is the optimal treatment? And while traditionally clinical improvements in reduction in size of the head and elevation of symptoms and signs have been used, some think that this is not sufficient. The question arises, persistent ventriculomegaly, does it lead to progressive neurological damage? Some authors have shown that accumulation of tau proteins can cause hydrologic damage going forward. Whereas others have propounded that brain volume rather than ventricular volume is the key to functionality. The International Infant Hydrocephalus Study, which started in 2004 and completed in 2013, was touted as being the first to provide a prospective comparison between the use of ETVs and shunts in infants with hydrocephalus. While we are with the five-year study results, the initial preliminary results suggest that shunting has superior early success rates compared to ETVs, although the success rate in both of them still said to be high. Well, while the IIHS study was commencing, we began to get literature from a certain gentleman in Uganda. In his setting, he started using endoscopic taut ventriculostomy. And even though initially was not very optimistic about its use, he found it to be valuable. The important thing is that the patient population here was predominantly infant and the etiology of hydrocephalus as opposed to those in Europe and North America was predominantly infectious in origin. Shortly afterwards, other literatures also came out. So, the same, Wolf also compared ETV with CPC, again, probably for the first time coming out. And the result here was that ETV combined with CPC was more successful than ETVs alone in infants and was being recommended as the best option of treating hydrocephalus in infants. Nine years later, a retrospective review by the ACRN Network also suggested that there's a safe procedure and recommended further study on ETV-CPC. They also highlighted a learning curve, which I will allude to later. So, how does ETV-CPC work? Well, if you are a proponent of the bulk flow model, what the ETV does is that it provides, bypasses the area of obstruction, either at the aqueduct of sylvia or at the exit foraminum, and assumes an intact absorptive capacity. However, some children we do know do not have that have insufficient absorptive capacity. And some workers have shown that arachnid granulations in children can be absent as up to two years. So, what CPC then does is to reduce the burden or the amount of cerebrospinal fluid that is presented to the patient. In under the hydrodynamic model, ETV creates a pulsation absorber by increasing the space between the subarachnid and ventricular spaces, whereas CPC reduces the intraventricular pulsations from the choroid plexus. Whichever model you choose, what it simply means is that when you combine ETV with CPC, it increases the range of infants and children of patients that can be treated endoscopically. We started combining this technique in 2007, and we published some of our early findings. And this talk will be about some of our more recent discoveries. I don't know what the audience is like, but just to go through quickly how we do it. In our setting, we turn the face of the patient to the left and prepare as if you want to shunt a child. And then, the covalent incision is made. And we use a flexible endoscope because our goal is to through one opening approach both ventricles and hopefully do the choroid plexus cauterization. This is a picture showing the floor of the third ventricle. That's the pituitary gland there. That's the clavus. And there is the basal artery in the floor of the third. We do not typically have access to further thick catheters, so we use the tip of the monopolar device, which is a bugby wire, and then we use that to make an opening on the floor. And then, we stretch that opening to create that. This is a video because one of the subcategories of children that we began to use ETVCPCs on in children with myelomeningoceles. And when you put endoscope on a child with myelomeningocele, the floor of the third ventricle is quite different. And this is the floor of the third ventricle in a typical child with myelomeningocele. It's very thick. Your usual anatomical landmarks are not there. But with a bit of practice and knowing where you're going to, you can actually still safely do an ETV. So, this is the infundibular recess there. And so, you just sort of go posterior to that without using any cautery. And gently tease the bugby wire through the various layers until you go behind the clivus there. That's the clivus. And then, have a better sense of where you want to make the endoscopic, the ventriculostomy. And there we are, just going forward a bit. All right. Okay. All right. And there we are. That's the basilar artery there pulsating. And there we're in front of the lilicous membrane there. All right. Also, a short video just describing what we do when we do corioliscoteri. Using the same device, that's a monopolar cautery device. The, you start at the feminine marrow and walk posteriorly into the body of the lateral ventricle. And then, with the aid of the flexibility afforded by the flexible endoscope, then you're able to get into the temporal horn and complete the corioplexis cauterization. And so, at the end, the corioplexis looks something like that. So, in the first five years, a total of 113 patients were treated endoscopically. We found out that over 66% of our patients are infants. Right from the beginning, we favored combining ETVCPC. So, almost 70% of the patients were treated with the combination. There was no significant gender difference. More than 60% of the patients were actually less than six months at the time of surgery. And this is important because these are age groups that are typically thought not to do well with endoscopic techniques. Of the 113 patients, only 24 patients failed, as at six months. And even when we followed this up for, the minimum follow-up here was at two years. It still stayed about the same. And that meant that we had a 78% success rate. When we compared what ETV, the success rate was slightly, the failure rate was slightly less, 20.5. And for ETV alone, the failure rate was about a quarter. Now, was age of any influence? Well, yes, because no failure was observed in patients over a year. And most of the failures occurred less than six months after the procedure. This is just a chart showing that, that ETV plus CPC did better than ETV alone. When we then further categorized into infants, only infants now, ETV CPC failure rate stayed about the same at 20.5. However, when in infants, ETV alone had an almost 50% failure rate. And again, most of the failures occurred in less than six months of age. This shows that if ETV alone is done for infants, then you just have about a 50% success rate. Whereas if you combine it with CPC, then your success rate is almost as high as 80%. In trying to determine optimal treatment, we did a quartz comparison between patients that had SHUNs and patients that had endoscopic total ventriculosomy combined with CPC over a six-year period. Well, the attrition rate was about the same. However, the mortality rate was 44% in the SHUN series and 34% in the ETV series. And that reflected obviously in the patients that were alive for follow-up going forward. The failure rate at six months was higher in the ETV series significantly compared to the SHUN series. So basically, our patient population is very young. And what we found out is that conventional ETV alone does not adequately solve the problem of hydrocephalus in these patients. We still SHUNs many of our patients. There are various reasons for that. One is the challenge of post-infective hydrocephalus, which is still not very amenable to endoscopic techniques. And also, some of the residents and younger attendings are not as comfortable with technique when it has to be done in the middle of the night. But our practice is now to offer a combination of ETV CPC as primary treatment, especially for children with, especially for infants with hydrocephalus. These are a few of our lessons learned. Equipment maintenance and the need for a flexible endoscope I think is important if one is going to do a complete and a thorough choroeplastic catheterization. Doing it with a rigid scope can be very challenging. So in conclusion, found out that ETV CPC is very useful in our patient population. The benefits of endoscopic techniques far outweigh any initial cost, and more attention should be paid to combining ETV CPC when treating infants with hydrocephalus. Thank you. A few. Thank you. So if I may just quickly acknowledge my colleagues at the Neurosurgery Unit in Luth, and then EIF, and also the Curehead International for the fellowship in which I learned the procedure and also that. And last but not the least, to welcome me to Abuja in July for the CAHNS meeting. Thank you. Thank you. Yeah, great talk. Any questions? Yes. Hi. Can you hear me? Yes. Hi, Jene Logan. I'm out of, I'm a neurosurgeon out of Nashville, Tennessee. I thank you for your presentation. I actually do a lot of medical missions in Nigeria, and I was just there about six weeks ago, and I happened to treat a lot of pediatric kids with SHUNTS. And one of the biggest crises there is actually the access to care when it comes to the cost. I do agree with you in that regard. My question to you specifically is, is there a significant cost difference, if you'd like to share, between placement of a VP SHUNT versus your CPC and ETV, and how that would impact the access, therefore, to patients who would require SHUNTS? And then the other question was, and it had more to do with the infection rate, for some of the failures that you experienced or have documented here with regard to the CPC and ETV versus VP SHUNTS itself, were you able to figure out which ones were actually post-op infections? All right. Thank you for the questions. Well, as you know, in Nigeria, patients have to do out of pocket care for health, and so the cost for the SHUNTS is about a third of the cost of care, at least in our practice. So that becomes a major hindrance. With CPC, they don't have to get the SHUNTS, you know, because the equipment is already there, and they just pay for the procedure and hospital stay, which is usually less as well. In determining the factors of infection, I don't have the data of that here, but infection was actually a major cause of increased mortality with the SHUNTS, actually. Thank you. Yeah, I'm curious of your failures that you went back over a year out, you know, from the ETV CPC. How many of those have you been able to salvage and reopen the ETV, or what would have been the biggest reason for failure, you think? Okay. So in the ETVs that failed, we were not able to go back in all of them, and we went back in like about five of them, about a quarter of them, and for some of them, the aqueduct was still open, and so we just converted to a SHUNT. We felt that that meant that there was some problem with absorption. In about three patients, the stoma had closed up, and so we reopened it, and they've done well, very well since then. Yeah. That's something I've noticed some of these kids going back a while out. The choroid plexus looked like it's totally regrown. It's pretty interesting. You know, you've cauterized it aggressively, and maybe you go back, check the ETV site, and you always look at the choroid, and there's quite a bit more there than I'd have expected. Well, I mean, actually, in fairness, most times when I go back, I don't see much, but every once in a while, you get caught out, and you're like, okay, well, maybe you didn't do as good a job at cauterization as you thought the first time. Yeah. Well, great talk. All right. Thank you.

Cerebellar Mutism in Adults

Endoscopic Thoracic Ventriculostomy

Choroid Plexus Cauterization

Hydrocephalus Treatment

Alternative to Shunts