Pediatric Neurosurgery and Research

Endoscopic Neurosurgery for Brain Tumors in Children

Phillip “Jay” Storm, MD: People come to CHOP neurosurgery from all over the world, and I think largely it’s because of the success we’ve had because we do such a team approach. It’s not one person, one surgeon, one physician. It’s that you’re getting a group of world-class physicians that all work well as a team.

Catherine Chiang, Lucas’s Mother: When we sat down with Dr. Storm, I think one of the things that made us feel so much more at ease was that he was a huge proponent for pushing for endonasal surgery.

Phillip “Jay” Storm, MD: The lesions we like to remove through the endoscope are ones that are midline, skull based lesions, tumors of the pituitary gland, the stalk, the hypothalamus, boney tumors, chordomas or sarcomas of the clivus or the bones themselves can be resected.

The endoscope is a camera that also has a light source on it so we’re able to see better inserting the endoscope than we can with a microscope. It gives us better access to the lesion. We usually have two monitors set up. So we’re looking across the table. The endoscope’s usually held by the otolaryngologist in one nostril, and I’m able to operate through the other nostril to remove the mass.

Catherine Chiang, Lucas’s Mother: When we sat down with Dr. Storm, we felt like he was competent, he was confident, and I think even Lucas said it himself that he just made him feel comfortable. And I think that’s where we felt like, here’s the surgeon that we really want to trust our child’s life with.

Roger Chiang, Lucas’s Father: Our son Lucas ended up doing surgery through the nasal, endonasal. But obviously the more traditional way was a craniotomy.

Nithin Asappa, MD: The traditional removal of these tumors were an open craniotomy, which essentially means, a child would have their head shaved here, a large incision over an area of bone where a bone flap, or an area bone would be removed, and then the brain would be retracted till they got to the area of the tumor.

The beauty of what we’re doing in these specialty selected cases is we’re identifying that that back of the sinus, the back of the sphenoid sinus, the tumor’s right there. So if we can access it through the sinus, we don’t have to go through these large incisions, this large removal of bone, that retraction of brain that all has long-term issues in our pediatric patients.

So, essentially what we’re doing is we’re taking a much smaller hole, the sinuses, where we’re using all of our specialized equipment, instrumentation and skillset, to identify the tumor and take it out in a very minimally invasive way.

James Palmer, MD: But you can’t just grab the tumor and pull because if you do, you pull some of the expensive real estate with you. It’s really more of a gentle tug. And then as we keep saying dissection, it’s our way of moving around that tissue so the tissue will roll out to us. That’s the process that takes hours.

Phillip “Jay” Storm, MD: When we do an endoscopic procedure, there’s always a neurosurgeon and an otolaryngologist. Oftentimes depending on the case and the severity, they’ll be two neurosurgeons and two otolaryngologists if it’s a very complicated case, or one that’s gonna take several hours. I actually have two of each in there.

John Lee, MD: Our goal is to remove the tumors completely. And that will give the child the best quality of life and the best possibility of a completely normal, long-term life. Now we’re able to come in endoscopically, do this wonderful surgery, and the patients leave without any deficits, and that is a 180 from what was happening before.

Phillip “Jay” Storm, MD: Generally speaking, when you do an endoscopic procedure, the patients have much less pain, and as a result, are able to leave the hospital much more quickly. They don’t have the swelling. They don’t have the pain, but they also don’t require any retraction of the brain. That’s one of the main advantages of doing this is we’re not even entering the skull as we have before, and we don’t have to retract on the brain itself.

Gregory Heurer, MD, PhD: We do over a thousand cases a year. One of the busiest, if not the busiest pediatric neurosurgery departments in the country. We also are diverse. We do a lot of different kinds of operations. But we do enough of them to be familiar, in the hospital, to be familiar with what we’re doing. So it doesn’t feel like you’re getting sent from one doctor to another. It feels like it’s a seamless set of doctors who are all taking to each other.

Todd Kilbaugh, MD: People that are dedicated to pediatric neurosurgery in particular, bring a level of competence that can’t be replaced. Having a board certified pediatric anesthesiologist who is also specifically trained in neuro-anesthesia and neurocritical care as a part of each patient’s delivery of care is incredibly important.

Phillip “Jay” Storm, MD: When we do these surgeries, it’s great to have a team that does a lot of them. And that’s why we’ve chosen the kind of a team approach. Just not only from anesthesia who do these cases, to the ICU, and to our oncology colleagues, this is a group approach, very collegial, multidisciplinary approach to treat all these patients from first time that they’ve been diagnosed with the tumor, and then their long-term follow-up is all done by a similar group of people that do a lot of these.

James Palmer, MD: There will be three of four of us scrubbed in at any time, sometimes even more, all bringing our skillset to improve the outcome. To go for complete tumor removal. To get that child back in action.

Catherine Chiang, Lucas’s Mother: Lucas is now three years out of surgery. He goes to school, he has fun with his friends. He goes on playdates. He plays sports. He fights with his siblings. He says no to me for every other thing that I ask him to do. He’s just a typical 11-year-old boy. That’s what makes me grateful. That’s what makes me happy. And also that’s what gives me hope.

Gregory Heurer, MD, PhD: We see our own daughters in those kids’ faces. From that respect, it’s harder. But I still wouldn’t do it any other way. It’s just the way you’re made is to care about kids. I’d rather have that kid be in there with me or with Dr. Storm, with people who care and people who are good at what they do, because we want to have as many kids have a good outcome as we can.

Phillip “Jay” Storm, MD: I think that everyone that we work realizes, I mean, what we do, we are privileged to be able to provide care to these children. And that means a lot to everybody and it’s a privileged and you have to earn it.

Moyamoya Disease Surgery for Children: Pial Synangiosis

Rebecca Ichord, MD: With Moyamoya syndrome there’s some kind of progressive narrowing of the two major arteries in the front of your neck, the carotid arteries. That narrowing gradually constricts the blood flow to the brain.

Phillip “Jay” Storm, MD: The goal of surgery is to provide a separate avenue to get blood to the brain.

Rebecca Ichord, MD: The child and their family who come to CHOP for surgery because of their blood vessel disease, their Moyamoya, or any other vascular disease, is going to experience what I feel is an incomparable team approach. And care that starts from the moment they walk in the door to not only after they leave the hospital but for years to come, for as long as they need it.

Phillip “Jay” Storm, MD: Moyamoya is the name that it was given because of the way it looks on the radiograph, like a puff of smoke. It’s a Japanese term. Moyamoya is a disease of the blood vessels. Usually you’ll see a narrowing of the carotid arteries, and then they often end up developing decreased blood flow to the brain, which leads to less oxygen to the brain, which then can lead to a stroke.

Rebecca Ichord, MD: A stroke is a clinical condition that is caused by a loss of blood flow or bleeding in a part of the brain.

Phillip “Jay” Storm, MD: It’s certainly something that we see plenty of. They can be having headaches. They may be having transient weakness. People may not be thinking is a result from stroke because they don’t think of children as having strokes.

Andrea Bancroft, Tia’s Mother: I thought it was a sinus issues and thought, oh I’m not going to leave the ER until they do a CAT scan to see what’s going on with her sinuses. It being a stroke, it just took me totally off guard. What 5–year-old has a stroke? I mean we all know how to handle an elderly person that has a stroke, but how do you handle a 5–year-old that’s had a stroke.

Within a few hours we were at CHOP, and Dr. Ichord came in. She first gave us the diagnosis, and was like, “Oh this is Moyamoya disease. Yes it’s very rare, but let me tell you about it.” And she knew everything about it.

Gregory Heurer, MD, PhD: I think that that level of sort of calmness that the whole hospital has with new or novel or unusual disease is helpful for the family because they can just see it as a health problem that we know how to treat.

Andrea Bancroft, Tia’s Mother: We talked more about the Moyamoya disease itself and what it does and what is causes and that there was surgery available. They talked about the surgery and that they had done it before. “It’s gonna be this way. And we’re both gonna be working on each side,” Dr. Storm, Dr. Heurer; it put my mind absolutely at ease.

Phillip “Jay” Storm, MD: The point of the pial synangiosis is to provide another access, another delivery system of blood to the brain with the goal being to prevent further strokes.

Rebecca Ichord, MD: You can sometimes talk about it in terms of say a traffic jam. And if one road is blocked, then you have to reroute the traffic through another road. So we have a set of blood vessels that go to our skull and our scalp and skin, and the surgeon identifies a viable branch from that external circulation.

Phillip “Jay” Storm, MD: We favor the indirect bypass at CHOP where you take a superficial temporal artery, which is in the scalp, and then you make an opening in the skull and literally just lay that artery over the brain. It’s quite loose, so literally just remove a piece of bone, and then lay that artery that’s quite free, on top of the brain. And then we put the skull back over top, notching out holes on either side, allowing it to come in, go down in the brain, and come back out into the scalp. Because the brain is trying to get arteries growing into it that little branches will start growing from that artery. And then you can see huge blood flow through the brain from that.

If you put a seed in the ground, and it’s going to start growing roots into it, then you lay this artery on top because it needs to … that brain wants to be fed oxygen, it will start growing into it to deliver blood and ultimately oxygen.

Yong Collins, RN, BSN: We tell a family it doesn’t usually cause much pain. Hospitalization is about maximum two to three days, and then they can go home.

Phillip “Jay” Storm, MD: You don’t get a benefit right away. It often takes three to six months, and then we do a repeat angiogram in a year just to see how the blood flow is developing.

I think one of the big benefits of having your neurosurgical care at CHOP is that all the other related fields are so strong. Neurosurgery is rarely a condition where you have one surgery and you’re done. I mean there are those, we have plenty of those, but in general these are complicated patients who need a lot of care, either neuro-oncology, neurology, endocrinology, physical therapy, rehabilitation, all that happens under one umbrella.

Lauren Krivitzky, PhD: The Pediatric Stroke Program at CHOP has expertise in caring for children with Moyamoya, including their medical care, surgery, but also sort of the long-term needs of patients, whether that is physical, psychological, school related.

Rebecca Ichord, MD: The Pediatric Stroke Program at CHOP was one of the first established in the nation and is viewed widely as one of the best.

Lauren Krivitzky, PhD: We see children with all different types of strokes and also other kinds of problems with the blood vessels in the brain.

Rebecca Ichord, MD: Stroke is just one part of neurovascular disorders. Many other kinds of abnormalities may affect the blood vessels in the brain. There are malformations called arteriovascular malformations. Another type is called a cavernous malformation. These and other kinds of blood vessel diseases can cause injury to the brain through bleeding, or through a risk of bleeding.

And so the Stroke Program in partnership with many other departments and with the surgery department take care of those kinds of problems together, as well as we take care of Moyamoya and the more traditional stroke condition.

Gregory Heurer, MD, PhD: We do over a thousand cases a year. One of the busiest if not the busiest pediatric neurosurgery departments in the country. We also are diverse. We do a lot of different kinds of operations. But we do enough of them to be familiar, in the hospital, to be familiar with what we’re doing. So it doesn’t feel like you’re getting sent from one doctor to another. It feels like it’s a seamless set of doctors who are all talking to each other.

Andrea Bancroft, Tia’s Mother: Every time we came there was either a social worker or the neurologist or someone was always there.

Michael Bancroft, Tia’s Father: Awesome, respectful, go out of their way if you need anything.

Elisa Olson, MEd: The most rewarding part is having the kids succeed, and seeing them come so far and work so hard and surpass, you know, the expectations that their parents have.

Andrea Bancroft, Tia’s Mother: I can be in the kitchen making cookies and she can play. She’s OK. I try to live every day and let her be a kid.

Rebecca Ichord, MD: The great thrill to me is just to witness, to bear witness, to how well children recover. How determined they are. How they find a way to be happy and whole, and that is humbling; it’s endlessly fascinating; and it’s very rewarding.

Choosing a Pediatric Neurosurgery Program: Advice from Parents

Jackie Radcliffe, Charlotte’s Mother: Diagnosis was probably the hardest part of the entire process. It’s probably the worst thing that can happen in life, is having something wrong with your child. So for us, definitely, diagnosis day was pretty much the worst day of our lives.

Catherine Chiang, Lucas’s Mother: From the moment the doctor said they did find something, I remember sort of kind of turning into this like robotic, you know, movement. Where, OK, and I just started writing down whatever the doctor was saying.

Jackie Radcliffe, Charlotte’s Mother: It was really hard in the beginning. I didn’t know where to go. I didn’t know any neurosurgeons.

Catherine Chiang, Lucas’s Mother: As with any type of rare illness, you’re so scared; you’re so petrified. Craniopharyngioma being such a rare tumor, we just started looking and looking, trying to find the best possible treatment.

Angela Waanders, MD, MPH: The first question anyone should ask their surgeon is, “How often have you done this?” And, “What is your approach?” And “What is going to be the goal of the surgery?”

Jackie Radcliffe, Charlotte’s Mother: I encourage other parents never be embarrassed. Always speak up. I remember when Charlotte was first diagnosed, it was hard for me to even make a phone call and ask a doctor, “How many times have you done this surgery on someone that’s 3 months old?” I look back and I’m like, “Thank God I did that.” So always speak up. Always ask questions. And yeah, Dr. Storm was always available to answer anything that we needed.

Phillip “Jay” Storm, MD: We are privileged to be able to provide care to these children. And, I mean, that means a lot to everybody. And it’s a privilege and, you know, you have to earn it.

Gregory Heurer, MD, PhD: I think that level of sort of calmness that the whole hospital has with new or novel or unusual disease is helpful for the families because they can just see it as a health problem that we know how to treat.

Andrea Bancroft, Tia’s Mother: It being a stroke, it just took me totally off guard. Within a few hours we were at CHOP, and Dr. Ichord came in. She first gave us the diagnosis was, “Oh this is moyamoya disease. Yes it’s very rare, but let me tell you about it.” And she knew everything about it.

Catherine Chiang, Lucas’s Mother: When we sat down with Dr. Storm, that we felt like he was competent; he was confident; and I think even Lucas said it himself that he just made him feel comfortable. And I think that’s where we felt like, here’s the surgeon that we really want to trust our child’s life with.

Jackie Radcliffe, Charlotte’s Mother: I felt very comfortable right away. Not only is he an expert and very skillful, he had amazing bedside manner. Not only did I look at him as a neurosurgeon, I looked at him as a human and a dad.

Gregory Heurer, MD, PhD: We see our own daughters in those kids’ faces. From that respect, it’s harder. But I still wouldn’t do it any other way. It’s just the way you’re made is to care about kids.

Phillip “Jay” Storm, MD: When we do these surgeries, it’s great to have a team that does a lot of them. And that’s why we’ve chosen the kind of very collegial, multi-disciplinary approach to treat all of these patients from the first time that they’ve been diagnosed with a tumor, and then their long-term follow-up.

Todd Kilbaugh, MD: People that are dedicated to pediatric neurosurgery, in particular, bring a level of competence that can’t be replaced.

Jackie Radcliffe, Charlotte’s Mother: Anesthesia is a huge part of anything. And it’s actually the scariest part for me. Another thing that brought us great comfort is that they are pediatric anesthesiologists, and they do this every signal day with small children.

Todd Kilbaugh, MD: Having a board certified pediatric anesthesiologist who is also specifically trained in neuro-anesthesia and neurocritical care as a part of each patient’s delivery of care is incredibly important.

Roger Chiang, Lucas’s Father: I definitely would recommend if other parents research all the different specialties within the hospital, number one being the neurosurgery, but all the other specialties that will affect their child.

Phillip “Jay” Storm, MD: I think one of the big benefits of having your neurosurgical care at CHOP is that all the other related fields are so strong. Neurosurgery is rarely a condition where you have one surgery and you’re done. I mean there are those, we have plenty of those, but in general these are complicated patients who need a lot of care. All that happens under one umbrella.

Catherine Chiang, Lucas’s Mother: The quality of life post-surgery is just as important, if not more important in a way. It’s been really, really nice having all of his care taken care of at CHOP with one visit; we’re sort of like all together. We’re all on the same page.

Jackie Radcliffe, Charlotte’s Mother: I felt so comfortable from the nurses to my social worker, who I still take to every single day. CHOP and Charlotte’s pediatrician, you know, are always in communication. And my pediatrician is always getting reports from CHOP. It does become your family.

Phillip “Jay” Storm, MD: Everyone’s at the same institution. And we’re all looking at the same data. And we’re all, in fact most of us are meeting once a week at conferences discussing their care. And I think it’s refreshing to the families to know that everyone’s thinking about their care.

Marisa Fabiszewski, Patient: I was diagnosed with a brain tumor at 13 years old. My surgery was on Feb. 14, 2007. I had a 13 ½-hour date with Dr. Storm. The care was phenomenal. It’s one of the reasons I want to be a nurse. It goes beyond a patient and care provider. It goes more to a personal level.

Yong Collins, RN, BSN: What I love most about being a pediatric neurosurgery nurse practitioner, honestly, is to see the patients and the families say to me, “My daughter or my son is doing well. And, you know, they’re going to college now. And they’re able to drive.”

Jackie Radcliffe, Charlotte’s Mother: My wish for Charlotte is to have a beautiful life, and she’s already inspired so many people. So I want her to continue to do that. And just to love, and be peaceful, and grow into a beautiful young woman.

Andrea Bancroft, Tia’s Mother: I can be in the kitchen making cookies and she can play; she’s OK. I try to live every day and let her be a kid.

Catherine Chiang, Lucas’s Mother: Lucas is now three years out of surgery. He goes to school; he has fun with his friends. He goes on playdates; he plays sports; he fights with his siblings. He says no to me for every other thing that I ask him to do. He’s just a typical 11-year-old boy. That’s what makes me grateful. That’s what makes me happy. And also that’s what gives me hope.

Jackie Radcliffe, Charlotte’s Mother: Have faith, have hope, and put your faith, put your trust in people who you choose to care for your child.

Pediatric Brain Tumor Research: Cures through Collaboration

Phillip “Jay” Storm, MD: For us, removing the tumor is only part of the job. We know that most of the brain tumors we remove, even with getting all of it out, which is certainly the first step to the best outcome, are going to come back. And we think our job is not just to remove the tumors, but also to find other treatments that are more effective, or at least less toxic.

Adam Resnick, PhD: The reason kids have tumors is often very different than the reason an adult might get a tumor or cancer. It’s not because they smoked, or were out in the sun too long. It’s because something went array. What goes wrong is unique for that patient. When you say brain tumor, you really mean a collection of diseases that are very heterogeneous, and complicated, and very different from one another. So kids’ tumors are not only very different from adults’, among them, among kids’ tumors themselves, there’s a great diversity of biology.

Angela Waanders, MD, MPH: Only recently did we start to unravel the genomic underpinnings of what is even the makeup of the tumors.

Adam Resnick, PhD: And so having a molecular understanding, having a detailed understanding of what it is that’s wrong within the cell, suddenly opens up doors to intervene in an entirely different way. There’s commonalities that we try to discover and discern. But we really treat each individual tumor as a unique entity.

Phillip “Jay” Storm, MD: What I’m most excited about right now at CHOP is our tumor consortium. To me that’s the future of tumor research. And that’s where we’re going to find the cures.

Angela Waanders, MD, MPH: It’s multiple institutions dedicated with a vision of collecting and sharing tumor tissue amongst research scientists.

Adam Resnick, PhD: So when Jay takes out the tumor, his focus is on taking out as much of the tumor as safely as possible. The minute that happens, the blood and tumor goes up to the pathology suite. The pathologist then begins to divide the tumor in multiple different ways to allow us to do the kind of research that we’re doing.

The tumors are frozen in liquid nitrogen at -200 degrees Celsius. They are snap frozen in a way that essentially stops everything at once. At the same time, pieces of tumor are frozen very slowly that will allow us to then take that tumor and keep it alive forever.

The tumor than comes to the laboratory where we then try to extract cells from the tumor. And it begins to give us a first view on how the cells really behave. And provides an opportunity for us to really study the cells as they are essentially dividing in a petri dish. The blood is processed to extract DNA, RNA and cells from the blood, and gets distributed across different storage systems.

Phillip “Jay” Storm, MD: CHOP as an institution and we as a laboratory have invested so much money in the infrastructure here to support the research from other institutions who can do the science and have great scientist, but maybe don’t have the money to do the sequencing. So we provide it for them.

Angela Waanders, MD, MPH: The goal is to share that information in real time amongst the whole community of scientists. And it’s annotated so we’re not just a scientist working in isolation. We’re annotating the tumor tissue with the actual clinical information.

Phillip “Jay” Storm, MD: They make all that information available to them at the same time as us. We’re not interesting in any kind of scientific advantage. We’re interested in scientific cures.

Angela Waanders, MD, MPH: This intersection of the scientists and the clinicians working together and having that combined information is really what’s going to push the field ahead.

Adam Resnick, PhD:  And so we began looking at these benign, so-called benign tumors. These were low-grade gliomas. And began looking at their genomes. And when we began looking at the genomes, we found that a very large number of them had only one thing wrong with them.

Phillip “Jay” Storm, MD: We know that these tumors look identical under the microscope, but have different genetic abnormalities. So now we, you know, check those tumors for whatever abnormality they have.

Adam Resnick, PhD: In a very short amount of time, we’ve gone from identifying, essentially, the broken part in the tumor, to initiating clinical trials. They’re no longer going to be treated simply as a patient that has a tumor that we’re gonna treat indiscriminately with a compound that’s just targeting rapidly dividing cells. We’re going to treat you as a patient that has a specific mutation in a specific gene with a compound that we think only targets that mutation.

Phillip “Jay” Storm, MD: So you want to know exactly what medication you’re giving and matching it to the genetic abnormality. The goal is to ultimately have that kind of information and power for all the tumors that we resect.

Adam Resnick, PhD: We never make the claim that what I do in the laboratory today will cure that person’s disease today. But many families recognize that research is an ongoing process with an unpredictable time scale. And many parents and families, I think, very soon will recognize how important is was to think about what happens to the tissue after it’s out. How will it impact the knowledge that we derived from that tissue towards the discovery process. And how it might help their own child, but for sure will help children in the future.

Phillip “Jay” Storm, MD: We’ve had patients in our lab working on the specific tumor that they have. And I think it gives it a real human touch to our researchers over there to realize that, you know, these patients are real. And these diseases are real. And the person next to them may be alive because of what they’re doing.

Marisa Fabiszewski, Patient: Over the summer I did research. I had the awesome opportunity to do research in Dr. Storm and Adam Resnick’s neurosurgery lab. And I was doing brain tumor research, which was very cool. Breakthroughs are happening. Breakthroughs are happening every day here at CHOP.