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SNI Digital, Innovations in Learning, a 3D Live video journal which is interactive with discussion and now it's offering podcasts on Amazon and Spotify soon to be on Apple under the heading of SNI
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Digital.
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In association with SNI Surgical Neurology International, a 2D internet journal
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is pleased to present Sam or El Baba MD who is the chief of pediatric neurosurgery and the president of the Leon Pediatric Neuroscience Center of Excellence at Orlando Health Arnold Palmer Hospital
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for Children and the professor of neurosurgery at the University of Central Florida College of Medicine in Orlando Florida.
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And heavy talking us today
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talking to US today about his work and fetal nerve surgery and being one of the unique Uh UH people in the world who's doing this work is Taco beyond fetal in Utero repair of my Woman Inca shell after
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the Moms Trial today We're talking with Samara Obama Simmers a professor of neurosurgery and he's at the Orlando Arnold Palmer Pediatric Medical Center is that correct Yeah on a Palmer hospital for
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children at Orlando health Okay and he'll tell us a little bit about his background today we're going to talk about two subjects one is his extensive experience in fetal neurosurgery where stands were
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sky and and and how likely this can be done and then you were going to talk about some endoscopic assisted surgery for Pineal region Lesions Re Yes, correct sir.
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Okay, great. Okay, the first topic, why don't you can share screen, we'll start. Okay. Thank you. Go ahead and, Sam, or go ahead and tell us your uniquely experience. There are only a few
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people in the world who really have done the volumes you've done and
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we're anxious to hear about it. Thank you, Professor Osmond. It's a great honor and privilege to join you and colleagues in this wonderful video series I'm glad today to share with you our
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experience in the first talk and feed the surgery in the other one on pineal region tumors in children.
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I would go over a few topics related to the field of fetal spand bifida surgery. The first thing I wanna talk about is where do we stand in spand bifida? First of all, as you know and most
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colleagues know if now all of them that spand bifida is a global disease and you don't do defects remain a global problem. We know the etiologists, multifactorial, some are genetic and
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environmental, and some metabolic, as you know, as folic acid supplements have been associated with decreasing the rates of incidence and prevalence worldwide. But overall, if we look at the
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world map, the numbers are still around one to five or one to eight cases per thousand pregnancies. What is the standard of care worldwide right now? The standard of care is to continue to perform
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the repair soon after birth, followed by a likely need for a shuttle, for treating the hydrocephalus. But the standard of care is changing in different parts of the world. If we look at the world
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map here, we see that Spanbetha remains a global problem. And in some countries like in India, for instance, the four to eight cases per thousand live births, This is an alarming number for high
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numbers.
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of spannabifida, and if we look at the external work being done at a global level to prevent spannabifida, unfortunately, only 23 of the world countries are doing folic acid fortification. So the
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main goal should be prevention, but how about the cases we're unable to prevent? You and I have done lots of repairs after birth. This is the standard of care as of today, but as I said earlier,
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this is slowly changing What is exciting right now in this field of fetal surgery? We all go back to the mom's trial, but before we talk about the trial, let's talk about what is fetal surgery.
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Fetar surgery is an operation where we perform a cesarean section on the mother, keeping her safe and a gel anesthesia, while the fetus is connected to the mother through the umbilical We're
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creating an artificial environment. for the fetus to stay warm and feeling as if it's normal in the anionic cavity. And the neurosurgeon would perform the repair for the spambifida, then replace
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the amniotic fluid and close the womb and continue the pregnancy. Why do we believe that fetus surgery is helpful? If we look at the benefits of fetus surgery, we'll talk about in a minute. Many
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of them has to do number one with the neuroprotection We all know that the amniotic fluid is considered in neurotoxic. So when we do coverage of the spinal cord early in pregnancy studies have shown
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also in animal studies that the neurological function of the spinal cord is better. And when we stop the fistula, when we stop the CSF leak at the site of the malamineus seal, all the studies have
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shown that there will be decrease in the negative pressure at the base of the skull. at the Fremen Magnum, and this leads to reversal completely or partially for the high brain herniation. And we
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all know that when the high brain herniation is better, the chances for obstructive hydrocephalus become less, which means the rates of shunting potential can become less. So there was an
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NIH-funded prospective randomized trial results were published in 2011.
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This in New England Journal of Medicine publication is free for download for any of us The primary outcome of the trial, randomizing a fetus to fetus surgery before 26 weeks pregnancy compared to the
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standard of care after birth, looked at two outcomes. The primary outcome was looking at mortality and rates of shunting at the age of one year. And the secondary outcome was looking at cognitive
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and motor outcomes at the age of 30 months. The trial had to be stopped early because of positive outcomes And if we summarize here. prenatal group, fetal surgery group to the left compared to the
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standard of care, postnatal surgery group to the right, we see almost a 50 reduction in the rates of shunting from 80 down to 40 rates of high-grade herning age from 100 down to 60. But most
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exciting was here also that the percentage of children who walked independently by the age of 30 months doubled, 40 were walking independently, and when they looked at the level of motor function,
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two levels below the anatomical level, they were statistically significant for improvement. Also cognitive testing should improve Bailey's scores and other cognitive tests, but obviously there were
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risks related to the pregnancy, like increased maternal complications, like preterm labor, half of the mother ruptured their membranes prior to end of pregnancy. And the most concerning part of
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the mom's trial was that 13
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of the babies who had fetus surgery delivered under 30 weeks of gestation. So the take-home message from the mom's trial is that fetus surgery for repairing spandobifida for open-year-old tube
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defects, either mild natal seal or maliscuses, if it's performed under 26 weeks of gestation, because almost
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near most of the patients would have a significant improvement in the high-brain herniation, which leads to significant reduction in the rates of shunting in half, as well as improved the cognitive
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function at the cost of increased rates of pre-maturity. Full-up studies from CHOP and other places also looked at other outcomes like urological function which showed improvement in the neurogenic
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bladder, but The game changer was around 2017, when ACOG, the American College of OBGYN, came out with a national guideline or recommendation saying that all pregnancies complicated by a span of
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effort that should be counseled in a non-directed fashion about all treatment options, including prenatal counseling for fetal surgery. And since this ACOG recommendation came out, the number of
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referrals for fetal repairs for a span Biffet in North America significantly increased.
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I have been blessed to work with amazing teams since the mom's trial results came out, first in St. Louis, immediately after the mom's trial, I worked with colleagues from the Turnafita Medicine
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and performing 60 cases before I moved to Florida. And now in Orlando, we have done up to today, 54 cases. And I've been involved in over 12 international cases. experience is around 120 cases.
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The inclusion criteria for being considered for fetus surgery are very strict, and we've followed very strictly the inclusion and exclusion criteria of the mom's trial. The indications should
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include open defects like
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myelominigoseal or myeloschesis, between T1 and S1 levels. There has to be evidence of high in-brain herniation, which is KR type 2 malformation, and the gestational age has to be under 26 weeks
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of pregnancy, and it has to be a documented normal kerotype or a microarray on testing
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of the immunofluid. The exclusion criteria are very long, and many of them, most of them, are maternal contraindications. As soon as the referral is received, go ahead. Can I start a few
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minutes and ask some questions? Go ahead. Go back to that That's right. I think it's key for our. Everybody was watching it so it's our between Tijuana and Aswan that Yes we're pretty much sure
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where we're going to find it the Hindbrain Herniation I you have to have evidence of Hindbrain Herniation KR A Chiari to have as a as a criteria for surgery yes hundred percent because this is how the
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circuit work as biodiversity the pathophysiology by reversing the Humbert Herniation trying to reverse it so the obstructive Hydrocephalus will get better then you will have improved rates for
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shunting so let me ask you about that a let's see what would be the reason why you wouldn't do ITU -T at a it was a oh they had an earlier stage of Chiari Korea where guesses carry one as it's kind of
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a debatable that So you want to wait until you want to wait for them to get signs of Chiari, which is Chiari 2. With that, obviously, and with leakage, the hindbrain settles into the foreman
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magnum. And therefore, it would be a fairly strict criteria, right? Correct. And the truth is, you know, I receive referrals almost in a weekly basis and evaluate mothers with foetuspan bifida
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as early as 18, weeks of pregnancy. The truth is over 90 of open Europe to defects wouldn't have very, very clear evidence of high-end pregnant herniation as early as 18 to 20 weeks of pregnancy.
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And we see it in ultrasound all the time. That's good. And so the next, that's interesting. The next one is that the pregnancy has to be less than 20, six weeks in development, right? Correct.
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And what's the reason for that? The reason for that is that when they designed the mom's trial in 2003, they had to depend on some pre-trial studies came out from Vanderbilt and CHOP. They looked
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at some sporadic cases that were done prior to the mom's trial, and they found that cases that were done after 26 weeks, it was too late, discovering the arachnoidal adhesions at the level of the
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craniservical junction where preventing the reversal of the cerebellar tonsils back into the Priscia fossa. And this eventually led to no improvement in
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the high brain herniation or the hydrocephalus shunting rates. In addition, that by repairing it after 26 weeks, the spinal cord has been exposed too long into the neurotoxic environment that the
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amniotic fluid, and there is no benefit for the motor function. those are really key things I that don't come out in the paper so I if you talk about that to others that's what those are really the
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key pieces of information or what I'm thinking I'm thinking I'm coming to another question in a minute but they have to have a a genetic or you could you do an amniocentesis Una analyzer genetically
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what are you looking for and why looking at carson groms you're looking for down syndrome you're looking for any tri -cities or genetic abnormalities that are associated with other congenital
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anomalies that can make the survey more risky so the trisomy is are contraindicated if the fetus has any genetic abnormality this needs to go in front of ethics consultation ethics board In every feed
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to surgery program, there's an ethics board that oversees our clinical operations that can help guiding us in case of any contraindications we meet during the counseling. Okay, terrific. Now, if
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I'm, let's say I'm
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in a area of the country, either high or low to middle-income country, and I do an ambient ostentesis,
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do I have to send it away to get these genetic analyses? It depends where you are in the world. At the time of the mom's trial, they were doing simple karyotyping or a fish. Now, we're doing
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microarray for most studies. And actually, it's a challenge because in the old days, we used to just do the normal karyotyping. You're looking for main trisomies and syndromes. Now with the
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microarray, We're seeing a lot of interesting micro deletions and. and then we have to do genetic counts consultations to see what's the clinical implication of that I can tell you that most genetic
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abnormalities we see in microarray outside of trisomy these are not leading to excluding fetal surgery after consultation with genetics because I would like to say most of us we have some genetic
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deletions here or there and with no clinical implications are smiling a little bit when you send the olden days the olden days for me are different than the all the path that led I would say outside
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up to twenty eight to thirty years ago are up to twenty years ago we used to do only carry typing notes microarray in other parts of the world it's carrier typing Yeah I'm Gonna ask your question now
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but you don't have to answer to later because I know the result you going to present are impressive But I'm always thinking about what's happening in the low to middle income countries. And I know
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we've developed a system, you've developed a system where, I mean, here you've been working and then you've got a hundred and some cases. It takes a lot of experience. It takes a lot of teamwork
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to do this. So we'll ask you a little bit later, how can this spread to other countries where this is now more common than it is in the United States? Absolutely At the end of my talk, I'll touch
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up on some international work we've been done and what are the resources needed. But the take a message is that FITA surge is not expensive. FITA surge can be done in almost anywhere in the world
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with minimal
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requirements for equipment or all what you need is building the team. That's the priceless
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component of any work we do. Okay, terrific, thank you I think it was a discussion at the end. Okay, Drew. Thank you. Show that image here, show it, tell us about it. Now that our furrow
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comes in, we start by doing the ultrasound, the fetal MRI. Here you can see Jim on the feet MRI, you can see here the
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malo ningusil, lumbusic origin, you can see here clearly, this is a 21 weeks of pregnancy, you can see here, even when the fetus decides off your hand, you see clearly the key arrow, do you see
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a gem? Yes, and you can see
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the ventricular legally as well. Amniocentesis is done by the metronophyte medicine team. Our program has been taking the lead and pride in doing 3D printing, which helps us understand the
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condition. One of the challenges in fetus surgery is that you operate in a patient you never met. So you go in and you look at a complex lesion that's you're facing for the first time So we worked
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with - with the three D printing engineers to produce almost near accurate model here you can see in this case then defeated a MRI this is the model and this is the enthralled with a photo and this
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helped us a lot also talking to the parents before fetal surgery this type of work neurosurgeons can do along we have to work as a team with colleagues for other specialties like Nuneaton EG obstetric
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Anaesthesia Maternal Fetal Medicine we do continuous ultrasound we do continuous replacement of amniotic fluid Ringer's lactate that's warned and this machine you can seat in any Anesthesia program
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worldwide otherwise there are no equipment needed actually after the fetal surgery was covered in in a minute but this is the only thing we use just reckless and amniotic fluid the mother comes in
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early in the morning you use my Sharpener to use a microscope Yes Yes We're coming in a second what can we do is that after the mother receives the epidural catheter early in the morning we do and the
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Jaren a seizure she has a lifetime position we do the surgery together and most cases from skin skin and the more cases we do the better we become at it and now I feel comfortable now as a
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neurosurgeon doing C sections and be obstetrician who he and I work together he feels comfortable assists in microsurgery though it's truly a great partnership now you can see here professor osman you
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can see here how the and our uteruses explant and this is around twenty five weeks
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and now we're doing with the Ultra sound mapping of the placenta as well as the localizing the defect of the spinal bifida then we do the hysterectomy with or without staplers and now we're looking
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with him under the microscope at the this or the uterine wall the amniotic membrane stapled and sutured to the uterine wall this tubing do you see this one replace amniotic fluid and now we're looking
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at the mile opening a seal here we can see the stretched skin and this is the true membrane that's covering an emerald Linga seal here are some of the more difficult cases the mildest cases or the
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flat open neural tube defects and they have some of the technical challenges we'll talk about them in a minute the technique evolved over the years since two thousand and thirteen we adopted a true
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Micro neurosurgical three layer closure by starting by during reachable Ization Arena relation of the placard followed by identification of Endura and achieving watertight closure with the graph
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followed by undermining under the skin over the muscles to confront chile and bring the muscle together. I give all the credit to my mentor, Professor Yashrager, who was my mentor in my early
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career when I was in Little Rock, and he was the main reason behind pushing the field into microneuris surgery. And in the MAMS trial, this surgery was not done with the microscope in most programs.
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Here you can see standing between the legs of the mother. She's in a lay thought of a position. My colleague, maternity, the medicine from the right side, an assistant from the left side. And
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this is one of the older cases when we used to do it with two-layer closure alone, with that tubularization of the placode. We can see here sharp microdice section surrounding the placode, then
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injecting some fluid under the dura, almost like a tissue expander, then elevation sharply elevating the dura so confidentially enclosing it primarily. But you can see here the spinal cord is flat.
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and he did lead to a near relation thus are some of the cases from the arms fourteen years ago I was sure now the new technique that we have adopted since then now in this case and this is a small
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defect I'm injecting now that an aesthetic into the buttock of the fetus the fetus is receiving in a seizure from their mother through the umbilical cord but for ethical reasons we're also giving the
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fetus a second round of Anesthesia into the Buttock
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we start by doing Sharp Micro dissection or another placard here's the spinal cord placard in the center doing Sharp Micro Dissection Circle freshly and excising all this ARC nor do membranes or skin
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attached and a placard and then injecting this fluid almost like a tissue expander to elevate the Dura then doing sharp elevation and a dear Sir confronting and basically elevating the dura of the
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underlying Lumber Sasha and paraspinal muscles it's like anatomy did not come to the medline it's a split to the side and now bring it together now we start with the first slayer which is retailer is
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HM Placard I do this bitch at around eight or ten o proline and now we're doing step by step Tomorrow's issue the placard if we look here can you see the diagram of a saucepan the top this is for the
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trainees watching us here can see that Sparkle placard is you continuous irrigation to keep the baby warm and now dissect if you look at the diagram here the the sharply dissecting the placard away
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from the Arachnoid and the skin next step if you look at the top left this is the Flap placard and now it's similar aroused and again now we do to the tumbler Ization the placard peer to peer and now
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after we completed the tubularized nation in some cases of defiance clearly tethered we also cut it after that we go for after we elevated the Dura with or without Lumbar Fascia we go for a watertight
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closure as much as possible and sometimes the legions go to the sacral region we need to augment this with a douro graft in some cases but the key is to achieve watertight closure so that there will
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be no more fistula a CSF leak the last step is doing the skin closure some we start with undermining circumstantially all the way to the flags on each side and we start by a this is the easy culture
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is a closure when the defect is small or we go with primary closure the skin unfortunately only half of the cases are easy the other half are medium -sized the effects selected three centimeters and
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at those cases we do inlay allow Derm graft which is a kind of vertical human dermis and you can see here we're doing mattress closure on top of the graph but what do we do with really complex wide
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legions like in this case this is a very wide mildness mile or sky says it's almost a five centimeter defect in those cases you have to use an ala Derm graft as an only after that's done we replace
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them now to fluid and we closed the warm and then we internalize the the uterus back into the retroperitoneal as face and after that's completed we monitor the mother and the hospital for four days
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she stays or encounter in Orlando for two weeks and then they go back to for follow up with their local physician we start by doing ultrasounds on day two it's almost like a spinal shock you don't
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actually do a spinal cord tumor they can have weakness in some cases here you can see and on day two and if a fetal hip and knee movements touch up moving and then by day two knee extension by Day
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three start seeing ankle into movement it's almost like descending improvement of the motor function are almost a sixty percent of the pregnancies will make it for an elective C section around a third
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seven weeks we do early functional assessment which is very important even the defects when there were wide we use grafts as skin will typically allies during the pregnancy on top of the graph in most
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cases after birth do not need to go back surgery and were able to treat them with a lot local wound care here are some examples of some recent cases the more experienced the surgeons will become the
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lesser the exposures are like in this case the whole exposure was around four centimeters and the microscope is very helpful so what happens now we did there appear like in this case on the left side
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at twenty five weeks now we do a follow up MRI then two weeks and you can see here beautiful skin growing over the dura and you can see the placard here in the center and most importantly we see CSF
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surrounding the craniservical junction suggesting reversal of the kyari. After the babies are born we do MRIs around six, 12 months and here you can see the conus will always be below but once we do
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tuberization we are re -
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we're putting together the the the thickness of the spinal cord back to where it should be and in most cases you'll see very nice see that it saves us around in the placard. When we compare the
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maternal, fetal and the unital outcomes compared to the mom's trial from a busy center like ours we found that the results are very comparable comparable and this applies to most busy fetal surgery
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centers the results as good as the mom's trial if looked better. When we looked at our cases I would just station my age at averages around twenty four weeks and three days the average neurosurgical
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repair times around forty minutes goes from twenty five minutes to an hour depending on the complexity of the legion but the whole surgery around at three to four hours an hour to set your age at
28:50
delivery is just to over thirty four weeks for four weeks and four days even better than the moms trial where zero maternal fetal mortality were perinatal mortality of three point three percent relate
29:04
to pre maturity and the mousetrap was at three percent and when we look at our kids are walking independently without devices at the age of three years they're at sixty percent other programs like a
29:19
charm penny and vanderbilt and many other places continue to work in improving the techniques like the group of thunderbolt they like to do release incisions on the side I'm sure you've seen them in
29:31
the past We don't like doing this technique but in some programs they they they like to do release decisions aside the primary closure of the medline the lead decisions on the side the openings heal
29:43
by secondary intention when we looked at the risk factors for
29:49
preterm delivery in our series we found a repair around or under at twenty three weeks was associated with higher rates of preterm labor so now we pushed most of our repairs around twenty four twenty
30:03
five weeks the truth is there's a learning curve in every program and the learning curve is around ten cases most programs they have their uPs and downs in the first then cases then the team and
30:16
experience will take off in two thousand and seventeen we publish our series look at the largest published series looking at he TV and his copic servants to last me
30:28
for those babies who prior for the surgery as I told you earlier the mom struck the Moms trial primarily looked a chance that we looked at the T V and we found that the risk factors for HIV equally
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failure have to do with the age when we do these as surgery after birth and usually try to push it beyond four months when we look now at our shunting rates in Orlando it's around twenty five percent
30:56
so let's go back if you remember the months trial was talk about eighty per cent shunting rate and the standard of care forty per cent in the fetus surgery group in the Moms trial in St Louis Our
31:09
Numbers were around twenty seven percent now we're under twenty five per cent shunting rate and I give this a credit to the E T V that in many cases it'd be successful what are the Red Flags for feed
31:21
the surgery the recliners we'd need to follow those kids long term because we are finding that the technique matters we take pride In pushing the envelope and doing my canary surgery for the repairs
31:35
many centers believed that a quicker repair should be enough the problem is we don't have enough data to discuss the long -term tethering the technology continues to advance many programs I can Brazil
31:48
and some centers in the US are doing fitness copier repairs without open hysterectomy but you can see here that placard is expose and a patches placed over the placard fraud by closing the skin a do
31:60
believe this is the future of photography but unfortunately right now it's considered experimental and there are some cum high rates of tethering of the spinal cord but the techniques to continue to
32:13
improve the some programs are pushing the envelope by publishing large sears fitness carpet outcomes the key is to share out the results with each other Ben Affleck reaches the North American Theater
32:27
Turkey network has a fetal immensely consortium where standards participate, and we share the data,
32:36
some data coming out, looking at outcomes, 10 years following the MAM style are showing that the results continue to look good, and we need to monitor those kids' long term. We're pushing, we
32:52
continue to push the envelope by making the surgery smaller and smaller. And the question is, as we make the histogram is smaller or smaller with the use of microsurgery, where the sparkly photo
33:03
repair can just lead to maybe one day a low-virgin delivery, and the jury is still out. What is the future? Some group in Sao Paulo, Brazil published FITA surgery for selected cases of encephalus
33:20
seals. No cases were done in North America yet as of today, but this is potentially something we can look at i see the surgery for some cranial conditions like in south muscles some programs are
33:36
looking at outside the box options like doing stem cells and they're still experimental but education is key and we we worked with colleagues from Brazil and anatomical simulators for the training of
33:53
performing surgery tracking the outcomes of those kids is very important watching them for tethering
34:01
is a very essential international collaborations are key as a measure earlier I've been fortunate to be involved when performing the fifth circuit in twelve countries and we are working with at least
34:15
ten more countries right now at different stages of preparations the key is having the right partnership neurosurgery maternal fetal medicine obstetrics and units allergy and a seizure you can do the
34:28
surgery in my opinion any country in the world where you have partnership you you can do safe Caesarean section anywhere in the world you can perform spotify to repair any word the world you just need
34:41
the team to be a collaborate collaborating following protocols here we performed a fetal surgery in Dubai and many other countries same conclusion Peter served as Guinea excitement and momentum
34:56
worldwide we believe that the technique matters and we believe we need to be tracking the data long term
35:04
Susan and I'm hoping that we will continue to be able to expand this worldwide thinking
35:12
may well it's a little some questions ah when you go to these other countries and you're doing a surgery I obviously have to have a microscope but you don't take anything else so much I assume you're
35:27
saying you can do this in these places and you're trying to establish teams are so right to recover from a cold okay allow me to call nine one one or something there that love God thank you okay I
35:43
don't see your picture you're recovering here yeah a pancake as some questions so your login first guy you got your team you're doing it here in the country I'll come back to that in a minute I'm
35:56
getting very good results now you're going to go to other places either in developed rural or the developing world I and you're establishing it and when you're going the only thing you take with you
36:10
is yourself in your bag or maybe a few micro instruments it but basically you can go and use our facilities their equipment and can can do the same same gets this same kind of results through the same
36:25
surgery is that a correct assumption AH to some degree but there are some steps ahead of that we don't go to perform surgery in any country in thirty minutes not invested to come first and watch an
36:41
operation with US or anywhere else though the way we usually engages then we start by Zoom Conference casual confess that conversation usually starts at a meeting somewhere then we do a zoom meeting
36:54
then we share with them our protocol which is like the Bible the the program have to follow specific steps for the protocol and then the team we always advise the team to come and travel and visit us
37:10
it's an open door for any program it needs some paperwork to get some listing privileges at our hospital or they can go to other fetal surgery center and after that after they watch one case they go
37:24
back to their country I finished the preparations getting the team together the protocols followed then would they do a mock case like making what is what do you do if there is an entrapped and
37:37
delivery you follow steps with a B C and D what do you do if there's any fatal hypothermia bradycardia you follow certain protocol steps and after that they start recruiting for the first case and we
37:52
do via zoom or Whatsapp in our screening for the first case a once a decision is made to proceed and the family wants to proceed then our team will travel myself and my Colleague maternal fetal
38:05
medicine and we help them with the first case and we scrub together and most programs they just need one case being joined by a consultant group in most places at one cases enough some places they ask
38:23
us to do two cases in the program takes all.
38:28
But traveling to visit another center is essential, in my opinion. I think it would make sense. Let's go through the natural history of the disease here. First of all, we had the introduction of
38:44
the use of folate. I remember this has got to be 20, 30 years ago, I was listening to a talk in Peru. And this was not an error searching. It was a fellow who got up and talked about the fact
38:59
that there was a higher incidence of mylaminega seal in the poor people compared to the wealthy people or the people who had good incomes in Peru And it struck me at that time, my god, this is an
39:16
environmental problem. And this is a major, you made a major statement there. Well, after that, Uh.
39:24
they got into folate deficiency in the diet and then we have countries that have adopted fortification with folate supplementation if an that's been successful are there other dietary deficiencies
39:42
that can lead to this defect or is it just not I'm not aware of any supplemental or proven supplements that are making a difference other than folic acid the key is not only prevention the key is also
40:02
preventing Stecher cases in the same family so if a lady now has a child with spinal Bifida the next time she is before she is planned to be pregnant for the second time she needs to take folic acid
40:17
and it has to be ten times the the preventive dose and this Is Something recommended by the WH ole to the keys not only prevention the key also preventing a second child in the same family as we We've
40:32
had a number or a number of videos with with the people in low to middle income countries recently with the Democratic Republic of Congo there is there's been an a spread of Monkey Pox I'M just going
40:48
to go aside from it and it turns out that a lot of that is a sexually transmitted disease and so forth but a lot of a lot of the things that can happen occur in low to middle income countries because
41:01
of poor sanitation and poor nutrition people have immune systems aren't well developed so it's it's like I'm driving a Mercedes Benz and to enter into rural Hung a demo Dirt rural Congo it's a wonder
41:21
and it's a miracle, but practically it's not sensible. So the key in some of these areas is to get the preventative measures, which is full-age supplementation. Is that pretty much accepted
41:32
worldwide and done? It is mostly, mostly it was some countries were skeptical, but the ISBN, International Society of Pediatric Neurosurgery, and many other societies last year attended a very
41:51
important meeting for prevention span, Bifid and WHO in Geneva. And they signed a declaration last year, pushing for the full-age supplementation worldwide. And the hope is that in the next five
42:05
to 10 years, we see the world map changing. Right now, again, up to the data from last year, only 23
42:14
of the world are actually doing supplementation. And that map you showed initially that would you. I would correlate with people whose diets were poor, who are obviously immunologically normalized
42:24
and so forth. Okay, so that's the first, it's an education message. It's a nutritional message. Now, and you and I were together, and I talk about this in Sub-Saharan Africa, a lot of the
42:40
people there don't have ultrasound, which isn't, so I'm getting to the point is, okay, we've got it, we're supplementing the diet, now we've got to detect the disease. And so obviously the key
42:52
issue here is you have to have something like ultrasound, which is non-invasive and certainly not harmful, is that becoming more widespread? Absolutely, the problem with us and your surgeons is
43:06
that we don't know what other specialties are doing in other countries. When I travel to many countries around the world and I go to the offices of obstetricians in India,
43:17
Even rural parts of India, I was shocked. Those guys are ready to defeat a surgery tomorrow. You just need the team. You just need the protocol. Everybody has an ultrasound. Every operating room,
43:29
they have the level one machine that replaces anionic fluid with warm fluid. Even in Brazil, in Brazil one time, they were not using the level one machine. They were using a bucket of sterile warm
43:44
saline and put in the amniotic cavity like a bucket. And it works. It works. Theta surgery is inexpensive. Theta surgery can be done, in my opinion, any country in the world. You just need the
43:59
buy-in from the team. And frankly, the buy-in from the maternal theta medicine or obstetricians, because they are the experts who would be maintaining the pregnancy after we finish closure, which
44:12
is key, maintaining the pregnancy its complications. and to the babies delivered needs a buy-in and commitment from partners in this team. But I am a believer and I've seen it in at least four
44:25
continents so far. This can be done safely and effectively all over the world. And if we talk about Africa very specifically, you and I have a webinar in the past talking ETVCPC, just think about
44:39
it this way. ETVCPC is an operation that came out of Africa, came out of Uganda, actually, as we discussed in the past. If you combine like a disease like spanabifida, when I saw cases in
44:54
different parts of Africa, spanabifida, they are heartbreaking. But if you combine the benefit of - first, you try to prevent expanabifida by supplementation-fluic acid. You try to lower the
45:07
numbers. Well, you lower some, but some you'll not be able to prevent. And then the ones who are a candidate, a facility to do it, you do fetus surgery. And even after fetus surgery, they
45:19
develop how to selfless. Rather than putting a shunt, you do ETV or CPC. Just imagine in 20 years from now, how this can be very, how can this be very impactful
45:34
for the status of curing, and not curing treating this disease that it's outcomes in different parts of the world. It's like a combo effect And you see, I'm going to go to a sign for a minute here
45:47
and then come back to the main talk here is, can you see the treatment of hydrocephalus?
45:54
And I'm living in 2100 now. And
45:60
we're now at 2025. So we're talking 75 years from now. Can you see using ETVs and
46:09
Coreaplexus correlation treat hydrocephalus in.
46:14
using the endoscopic surgery, intrauterine in those countries as a future treatment?
46:23
I have to be skeptical in my answer and say the final SOB in two years. So now when we get the results of the ST trial, the NIH funded prospective Adondomy trial where now I'm year three out of five.
46:38
And I think in my opinion, the results more likely than not would be favorable. And then in my opinion, if a prospective Adondomy trial is showing us very long-term trusted, durable outcomes for
46:54
ATV CPC, I do believe this should
46:58
go worldwide and the rates are shunting, we should drop them significantly. I don't want to have to see ATV CPC done worldwide if I don't have long-term data to support it, but I. I'm cautiously
47:12
optimistic that this would be very favorable from the trial, and this would be the future. Well, one of the things I want the audience, our viewers to see is not what you do today, but what do
47:24
you think is gonna happen with his disease in the future? So now we'll get back to this, we've got the patient, and one of the things that they need to have is an ultrasound. We were on a call
47:34
where some of the people didn't have them, others did, and they found it was very useful It's about5, 000 in low to middle income countries, and you could tell if they had a subdural hematomas in
47:49
a ventricular hemorrhage. I would imagine you could use the same instrument with a different
47:54
tip to check what's going on in the uterus with the baby and the fetal. So it's an investment in other countries that has wide applications, and therefore the cost per case is becomes low. Is that
48:09
correct?
48:12
So now we've got to the point where you've made the diagnosis, we've got to, we've found a way
48:18
to do it non-invasively with the ultrasound. When is it early as you're gonna pick this disease up?
48:25
You talk about the spambethano, correct? Yes, right. And it depends which part of the world you're in. Here in North America, most of anatomical screening ultrasounds are done around 18 weeks So
48:39
these are furlums and furlums. When
48:43
does it appear?
48:47
I mean, there are some studies showing that as early as 15, 16 weeks pregnancy, you're able to see the defect and you can see the hindering herniation. So we've got it. Most of logistically, or
49:01
when we talk about the, what's the common practice worldwide around 18 to 21 weeks? So I'm establishing we've got a narrow window here. If you want to do this for four, 26 weeks, and it's
49:15
generally, let's say may occur at 15, you've got
49:22
11 weeks here. That's two months, three months, and so you don't have a lot of time, you have some time to work on it and get people prepared, but it isn't like you've got nine months to work on
49:36
this. That is correct. And what's happening is, let's give you just a quick example. Most cases come to us around 18, 22 weeks. By the time they come to us, amnocentesis is not done. Results
49:50
take typically seven to 10 days. FETAL MRI needs a few days to schedule. We need to do FETAL echo as well. We need consultation, the neurosurgeon, and the maternal FETAL medicine.
50:03
So you need a coordinator for this program. You need a logistics people who will do entrance preauthorization, they do the. coordinator and getting stuff done. But so far, we have failed zero
50:17
times in getting the surgery done before 26 weeks. The team will pull forces together and reschedule my cases around to make them work with the rest of the team until we get it done. We have the
50:30
clock is ticking and we need to get it done in a timely fashion. So now you've introduced another factor and that is not only you don't need to take any special equipment, but you need to have a
50:43
team that's educated, that's trained. And then it tells us if you're going to do this, it can't be an occasional operation. So if you're in a low to middle income country, it would be a
50:54
reasonable idea to establish a center where you could attract these cases. You could have a country like Kenya where they could have maybe several spots or Nigeria or
51:09
something. So you would suggest that you at least try to pick a place where you can centralize the care, at least initially. Would that make sense? That makes sense. Okay, let's go to the
51:24
surgery. So you've got your colleague, you go ahead and do the surgery. Positioning, positioning becomes important, doesn't it? Say it again. Positioning becomes important in terms of the fetus,
51:37
in terms and where the fetus is lying in the placenta. Is that correct? Absolutely. The fetus back has to be positioned in a way to be
51:52
near the incision that you're planning and the incision has to be far from the placenta. So we do keep mapping with the ultrasound until we find the sweet spot where it's far from the vessels and the
52:06
placenta and the back of the fetus is as close as possible. And you use the amniotic
52:14
fluid and fusion, which I saw in one of your videos there, it was just constantly pouring in the field to provide the natural environment and metabolic environment, that correct? That is, yes,
52:26
it's a warm ringer's lactate and we mix it with vancomycin to reduce rates of infection. Okay, now let's say we've gone through the surgery, you've done the surgery, you've been able to manage the
52:39
small defects and the large defects, the closure becomes important because otherwise you wind up with a CSF leak and you're back where you started from in some ways. So you've got to make sure it's
52:50
watertight.
52:51
Yes,
52:53
there's a controversy about the importance of watertight closure. As I mentioned, you used some programs, including from the mom's trial, they used to close them in utero, close fan buffettas
53:05
with just a quickie stitches on the skin, even without closing the gear on the outcomes were good. And the question is, do you really need water-tight closure, even to prevent a pseudo-mingucile,
53:16
or just close in the skin is enough? It's controversial in our community, but we believe in meticulous microneurosurgical reconstruction of the fecal sac and getting a water-tight closure. And this,
53:31
in our data, I didn't talk about it today, this can lead to reduce rates of tethering in the future And it also would seem to me that if one of the purposes of this is to wait till somebody have
53:43
somebody who has a Chiari II, which only comes because of the spinal fluids flowing out from the spine of Bifida, it would make sense that you should probably have as tight a closure as you could
53:55
because that's one of the reasons you're doing the surgery. That is correct. Okay. All right. Now we're finished with the surgery. What are the complications? The complications to the mother
54:07
from one of my reading, as you can get.
54:11
delivery preterm, I don't know if they're still burst. What are the complications? Do you have to have an open Caesarean section? What do they have? What are
54:22
the other parts of it? The most common complications are basically interrupting nature. You're opening and suturing amniotic membrane when you should not be doing that. So anything is related to
54:37
the interruption of the pregnancy. So the tendency of the uterus after surgery wanted to expel this maybe. So
55:03
what do we do in the first few days? To a colitic agents, reduced contractions, pain control. And after leaving the hospital monitoring for ruptured membranes, ruptured membranes happen to get
55:04
admitted to the hospital until they deliver. The pre-term labor, ruptured membranes. The risk infection is extremely small. In this case, we'll get infected unfortunately lead to bad outcome,
55:12
which is early
55:18
delivery. And one of the main long-term complications of this surgery is uterine rupture in case if the future pregnancy would be less than two years after the delivery of this feata surgery baby.
55:32
So we make our mothers and their consenting and counseling process, Garrett, make sure that there will be no pregnancy for two years after this delivery. So not only we establish that we have to
55:47
have nutritional support, we have to have an ultrasound in our way of making the early diagnosis. We have to have education. You have to have a team of people together to do this. You have to have
56:03
prenatal care. I mean, these are all things that don't happen. You have postnatal care and follow, right? So this is just a surgical procedure.
56:11
This is a treatment of a disease process. This is a serious business. This is a serious intervention and two patients at the same time. It has to be done by following meticulously all the protocols
56:26
working as a team with other specialties and deciding about who are the right patients to go through this operation. We do also in the other psychological counseling to make sure the mother is ready
56:38
to go through this journey. Okay, and the last question about this before we get to the next one, and I got that from, I'm gonna just share with a minute here. I got this from
56:51
a paper you probably know about. This paper was a review. I thought it was a very good paper. I'm sure you're aware of this. On the review of spinal malformations and fetal surgery Ahh. just for
57:09
the audience. And one of the things we'll need to have is a, is a list of, a short list of references that you can give to them. We'll add this to it if you think that's worthwhile. So the last
57:19
question I want to ask you is, I'm going to stop sharing here. The last question I want to ask you is now we've talked about 2050. By 2050, I would expect that this fetal surgery is going to be
57:33
more widespread and more worldwide. I would also
57:40
expect that a biochemical treatment of it, which is with folate, is going
57:48
to be also worldwide, which means the incidence of the disease should
57:54
decrease and the pediatric population or the infant population is going to be very high in Africa, in Southeast Asia. says some degree in South America in the developed world, the birth rate is low.
58:12
So we're talking, if we're looking into the future, and we're going to be treating the disease, the diseases are going to be in the low to middle income countries. True. Correct. All right. So
58:24
you've traveled around the world, you've seen everything, anything we didn't cover that you want to add. I think we covered it well after all colleagues who listened to this conversation talk we
58:36
had today. A very welcome to reach out to me and answer any questions I may have. We are always involved in discussions with colleagues from around the world. Sometimes we start by doing Zoom
58:52
conversations. The key is not near surges, the key is the team. Finding the right local partner who's willing to go on this journey with you looking for to support colleagues anywhere in the world.
59:06
Well, you've had a very, very major concept in healthcare. Healthcare isn't getting simpler, it's getting more complicated, and it's getting more, more sub-specialized. And one of the things
59:18
it's hard for people to understand is that you do have to work in a team. Well, now if you're working in a team, the issue that comes up is, well, what's the compensation?
59:30
And so this is a problem that's gonna confront people as we move into the end of the 21st century here, because medicine's going to be delivered by teams of people, just like you've talked about,
59:43
specializing in areas where we can get expert people and good results. But since I agree with you, I think we covered it pretty thoroughly. I think the answer that they need to get out of this is
59:54
this is a disease you can treat. There are lots of steps to do this. You need to associate yourself with somebody who's experienced and then go from there with the team. And then you're gonna have
1:00:06
the highest chance of being successful after it gets going. Absolutely. Okay. That's correct. All right, I think we'll close here. I think you did a terrific job. And I congratulate you on what
1:00:18
you've done. This is revolutionary surgery in a lot of ways. When I was born, or when I was going in a neurosurgery, not too much difference, 20 years, we would close these primarily and they
1:00:34
would deliver and that's what it was. Today we've got folate, we've got intrauterine surgery, we've got ultrasound, we've got microscopic surgery, a major, major change in the treatment of this
1:00:48
disease and we're on the cusp of treating hydrocephalus.
1:00:53
The last question, I said the last question before, are we gonna, is this disease gonna be treated by 2100 by pre-determining genetically what the problem is can either reversing it or.
1:01:07
or giving them some Asian that stops the disease? I think there are
1:01:16
some genetic or proven genetic association with spina bifida. The key is prevention, in my opinion, potentially we may be able to near eradicate this disease by prevention worldwide But I cannot
1:01:34
under emphasize the importance of
1:01:39
targeted therapy. You never know, in 100 years from now, we know it's the gene for spina bifida and they can
1:01:49
knock out this
1:01:52
gene in the mother before she gets pregnant and this disease will be eradicated 100 like polio one day I don't know.
1:02:06
Well, you've been involved in a number of very large scale studies. I think one had 1, 000 patients from multiple centers worldwide, two of them, in treating a tumor starting from edge of a
1:02:18
blastoma and then a
1:02:23
pin to moments and so forth, where now they're being subcategorized and getting various genetic stages. And we find this in gliomas Also, we have targeted therapy that stops the disease.
1:02:37
Absolutely, the future is so bright for giving us healthier children who are the leaders for tomorrow. Children now, we're treating their tumors. There will be a day we're going to prevent their
1:02:53
tumors and it's going to take some time but we're heading in that direction. Well, terrific, Shamer I think we've told everybody. at least where we are now, where we came from, and where we're
1:03:07
going. And we've also told them how to get there. Yes, sir, thank you, thank you. These are the key references in the presentations that you will see. Take screenshots, be prepared to take
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