Dr. Greenfield: At its most basic level, Chiari malformation is a terribly misunderstood condition.
Erin: Dr. Jeffrey Greenfield is a neurosurgeon at NewYork-Presbyterian and Weill Cornell Medicine. He specializes in pediatric neurosurgery, specifically, treating patients with a rare congenital brain condition called Chiari malformation.
Dr. Greenfield: The easiest way to think about it is a mismatch between the size of the skull in the back of the head, the posterior fossa, a mismatch between that compartment, structurally, and the size of the cerebellum itself.
Erin: Because of that mismatch, the cerebellum begins to compress the brain stem.
Dr. Greenfield: Even subtle compression in that area can be, uh, tremendously disabling.
Erin: Although most pediatric specialists are trained to only treat children, over the years as he treated patients for Chiari malformation, Dr. Greenfield kept encountering an interesting request.
Dr. Greenfield: We started finding families that had Chiari, and so I would operate on a child, they'd have a good outcome, and then at the follow-up visit, the mom would be like, "Um, by the way, I've got this too. Can you take a look at my scan?"
Erin: These adult patients had never received treatment for their Chiari symptoms, despite having the condition since birth.
Dr. Greenfield: They get lost between the expertise of pediatric neurosurgeons being centered at pediatric-only hospitals and adult neurosurgeons who are very uncomfortable taking care of these congenital malformations. Very, very few pediatric neurosurgeons find themselves at an institution that can really support this type of an endeavor. Because NYP is an integrated pediatric adult hospital, I've been able to find this really cool and interesting niche of patients all over the country who can't find pediatric-trained but adult-capable neurosurgeons to take care of these inborn problems. It's why we're all at places like NYP and Weill Cornell so that we can, you know, push the envelope a little bit further and improve the care for our patients.
Erin: I'm Erin Welsh, and this is Advances in Care.
Today, I'll speak with Dr. Jeffrey Greenfield about how he is pioneering a transitional care model through the Chiari Care Program at NewYork-Presbyterian and Weill Cornell Medicine, creating new avenues for treatment and offering renewed hope for patients with Chiari malformation.
Dr. Greenfield, thanks for joining us today. It's so great to have you here.
Dr. Greenfield: Yeah, it's really a pleasure to be with you today, Erin. Thanks for inviting me.
Erin: Just to start us off, I'm so curious about your journey to pediatric neurosurgery. What was that process like of discovering this is the field that you want to go into?
Dr. Greenfield: I found myself in the realm of the neurosciences from a very early age. I was a neuroscience major at Amherst College. Uh, I was doing experiments on mice on learning and memory, um, from the very early age, not knowing exactly where that was going to lead, but knowing that I was fascinated with the brain. And then when I was looking into subspecialties in medical school, I was really looking more at the neurosciences that were non-surgical, so psychiatry, neurology. I was still fascinated. I thought I was gonna do research, and the former chair at Weill Cornell, we had a chance conversation about research, and he invited me out to the neurosurgical service for a month. And by day three, my medical student friends all told me that I had found what I had wanted 'cause they had never seen me happier or more fulfilled.
Erin: Oh, I, I love that story.
Dr. Greenfield: Yeah.
Erin: And so it was sort of this chance encounter, meeting the right people at the right time, being open to this, this field, and then where did pediatrics fit into that?
Dr. Greenfield: The procedures, um, and the anatomy drew me in initially, but then the patient care and the nuance of taking care of kids and their families, it, it ended up being a really nice synergy between kind of the, the part of my brain that wanted to do psychiatry and sit and talk to patients and the part of my energy that wanted to be in the operating room operating.
Erin: And you have spent the last, uh, few decades building this renowned pediatric neurosurgery program at NewYork-Presbyterian and Weill Cornell Medicine, and one of the really crucial hallmarks of this program is that it provides this transitional care. Can you tell me more about what goes into building a, a program like this and why it's so important, especially when it comes to congenital neurological conditions?
Dr. Greenfield: Yeah, I, I think it's been a little bit of an unexpected twist. And so I see a combination now of actually pediatric and adult patients with the common link being that they have some type of a congenital neurosurgical problem. The will to go beyond the envelope of what pediatric neurosurgery typically serves can be challenging. You know, NewYork-Presbyterian is an integrated hospital, and so it allows us the luxury of I could operate on a two-year-old, you know, in the morning and a sixty-year-old in the afternoon in the same operating room. I think that's really a unique thing that our hospital provides, but there are logistical things in terms of h-how to operate on two different age patients. And as I started seeing more adults in my pediatric practice, there were real learning curves that we had to address, namely with the staff not feeling comfortable taking care of a 40-year-old or a 50-year-old. You know, we didn't have to do pre-op cardiac clearances on five-year-olds, but on, you know, a 60-year-old, all of a sudden there's a different skill set. We just co-authored a whole journal issue on challenges in transitional neurosurgery, and the traditional challenges have been around taking an 18-year-old who's lived with something their whole life and finding an adult neurosurgeon to take care of them. All kids need to transition to adult providers. Um, this is just one really big niche where, um, adults tend to get lost.
Erin: Yeah, it's a big gap in, like, the continuity of care. And backing up for just a minute, can you tell me a little bit more about Chiari? Like, what does this condition look like in a patient of any age?
Dr. Greenfield: Whether it's referred to as an anomaly or a congenital malformation or often thought to be just a completely incidental and normal finding, as I've learned, um, varies depending on who you talk to and who the patients have seen. But it has led to there being a very...common and unfortunate delay in medical care being obtained because so many people are told that it's incidental or not related to their symptom presentation. The radiology and the, uh, symptom complex don't often match up, and that's part of our, our conundrum here. And so it's often thought of as related to the degree of herniation of the cerebellum outside of the skull. And so as the skull and upper cervical spine develop, they become a little bit, um, atrophic and can't contain the cerebellum as it grows. And so the cerebellum, which, you know, millimeter for millimeter contains more neurons than any part of the brain, literally fungates out and herniates out into the s- into the spinal canal. And so who is symptomatic and who is not, it's the degree of compression of the brainstem. And the brainstem, where the cranial nerves emerge, controls almost every single feature, autonomic nervous system, cranial nerve nuclei, and all the tracts that connect the brain with the spinal cord. And so even subtle compression in that area can be, uh, tremendously disabling.
Erin: What does that look like? What, what brings somebody in to be tested?
Dr. Greenfield: Most patients will have a constellation of a few common symptoms. At the top of the list is always exertionally related headaches. There are patients who have had a headache every time they sneeze their entire life, not knowing that they have a Chiari, and that's their only symptom. And really common symptoms are dizziness, tingling in the hands and the feet, nausea, ringing in the ears, difficulty swallowing, sometimes sleep apnea. And it... And the common feature for all of these is some of the fibers that underlie those activities are located in the brainstem. And so when someone has 20 different symptoms, the two options are, according to the patients, people tell them that they're crazy or that they're making it up, or you find a common neurologic pathway that makes sense. And it's often the brainstem, which is the common denominator for all of these patients, where they've got so many symptoms that you think it can't possibly be real until you realize that they're all focused on this one little tiny piece of real estate in the back of the brain.
Erin: Right. And then how do you determine the right treatment approach?
Dr. Greenfield: The decision-making about surgery is, I would say 90% of the time is based on the symptoms, about the quality of life, and about how those symptoms are affecting the individual. We operate on a really small percentage of our patients. Um, the vast majority of them we send back for medical management and neurologic care. We, we view Chiari malformation surgery as the last stop on the train. Like, you, you are gonna exhaust all the non-surgical options before we get there.
Erin: And can you tell me more about some of the factors that make treating Chiari malformation in adults a bit more challenging?
Dr. Greenfield: The biggest challenge for adults in a Chiari clinic is that there are a lot of things that mimic Chiari that can be very, not only confusing diagnostically, but also really dangerous to miss. One is, uh, something called a cerebrospinal fluid leak. And so if you have a spinal fluid leak somewhere in your spine, the actual manifestations on a brain MRI scan may be really similar in lots of ways to a Chiari malformation, meaning that the back of the brain may droop down. The cerebellar tonsils can actually herniate out. They'll have headaches. They'll have ringing in the ears. They'll have a lot of similar symptoms. And what happens if you do Chiari surgery on these patients, you might imagine making the hole bigger in a patient whose brain is being pulled down actually makes things worse. We've seen this really interesting population of patients with CSF leaks and have been, you know, really thrilled that the opportunity to build a CSF leak center has been really kind of parallel with the Chiari center here. And Gayle Salama has done a tremendous job in neuroradiology building this world-class CSF leak center. And so we collaborate on a weekly basis and now fix CSF leaks as the primary cause for this brain herniation, when in the past I think this was missed the vast majority of the time. I see these patients from all over the country who have had the wrong surgery without having the proper workup ahead of time. And so the challenge is really on the diagnostic side and how it's really opened up this whole window into how Chiari is really being viewed as a multiple different etiologies that all present similarly with compression of the brainstem through a myriad of different factors.
Erin: And I think that everything that you just touched on from proper diagnosis to treatment really just speaks to this profound need for an interdisciplinary transitional care approach. And, you know, you mentioned the Chiari Care Center. Could you tell me more about what the Chiari Care program offers?
Dr. Greenfield: It was important to have lots of pieces in place so that we could take care of the patients no matter what it was. And so for me, it was, you know, everything was greater than the sum of its parts. Like, building the program was only gonna be successful if we had the pieces in place and really great colleagues. And so I've been fortunate to have been here long enough that I know who these, like, golden pockets of medical care and surgical care are. And so I sort of built all these little pieces from ophthalmology, urology, neurology, in, in addition to the, the surgical subspecialists. So if you come to me for care, you may not end up with surgery, but you'll end up with at least a roadmap and a diagnosis and a, like a direction to say, "This is what you need."
Erin: That must provide such relief for these patients who have potentially gone years trying to figure out what's going on and not getting the help that they need. And so now with all that data at your fingertips, are you using tools like AI to help sort through it and hopefully predict patient outcomes?
Dr. Greenfield: Yeah. I think that's maybe, you know, the most exciting part that kind of tickles my scientist side. We just had our 1,000th surgical patient, and that's exciting from, like, you know, success standpoint of the program. But what's more impressive is that we've been able to collect data on all these patients, and we have MRI scans from 1,000 patients, and we have outcomes data.We have some really fantastic scientists now adept at machine learning and artificial intelligence who are now plugging these thousands and thousands of scans into models that are going to hopefully help better predict who is gonna be a good candidate for surgery and who may need another route to treat their symptoms. That will be really powerful for patients to have that data in front of them, um, and referring physicians when we're trying to make those really tough decisions.
Erin: I mean, that would be a, a huge step forward in terms of guiding treatment, and I imagine this wealth of data will also open up some impactful new avenues for research as well. Are there any research projects you're working on right now that you're excited about?
Dr. Greenfield: Yeah. For years and years, we heard from our patients that they had brain fog as part of their symptom complex, and I think that there is a real cognitive component to Chiari. There's some really e- exciting work being done on the cognitive connectivity of the cerebellum with the cortex. It is a really, really common feature of very young kids to have some language delay with Chiari, and really, really common with older adults to have word-finding difficulty, spatial, um, awareness difficulties, short-term memory issues. And so our group started picking up on this and started doing some research into this and doing some neuropsych assessments of patients, finding that there was some significant delays and deficits in very specific areas of the brain that were common in Chiari patients. And so we wrote this review looking at all of the evidence and all the literature around the country from multiple medical centers as the background for a clinical trial, which we've submitted to the IRP, which is actually preoperative neuropsychologic testing and postoperative neurologic testing so that it's not just the patient reporting to me, "Yeah, the cobwebs are cleared," but rather real discrete data from neuropsychologists in our department who are s- really engaged in this to give patients one more piece of information as to whether or not some of their symptoms beforehand might be impacted by surgery.
Erin: What impact do you hope this research might have on care for Chiari patients?
Dr. Greenfield: I think what it's showing is that there is a lot about the cerebellum and the connectivity to the rest of the brain that we don't understand, and there are several other areas of investigation that I'm really interested in that I think have been largely ignored due to just our biases of believing what we've been told in terms of how the brain circuitry works. And so I think it's an exciting opportunity to push the envelope a little bit more in terms of trying to link some of the, the scientific side and the academic side with what we're doing in the clinic. And again, that's always the goal of what we're doing in academic medicine, so it, it, it feels really natural, and I think it's gonna be really fruitful coming forward.
Erin: Right. And it's the fact that so many patients are coming to your center from all over the country that makes these research advancements possible. I know that just recently there was an article published about a family who came to your Chiari clinic from West Virginia. You treated their little girl and then ended up treating three of her sisters for Chiari as well. I would love it if you could take me through that story.
Dr. Greenfield: Yeah. The, I mean, they're a fantastic family, and there are amazing cities like New York where you can't, you know, you can't walk a block without stumbling into a neurosurgeon. But there are also big, vast swaths of this country where it's really hard to find not only, you know, excellent care, but specialized access to this type of, you know, niche neurosurgery. And so listening to these two parents who were so patient and so kind, and every time they brought me another sister, I said, "You gotta be kidding me. There's no possible way." And every single one of them had real pathology. And clearly, you know, at a certain point it made really good sense that there is a genetic link. Of course there is. This is a neurodevelopmental disorder at its basis. They are now one family in our genetic study, which is recognizing that these are not just all one-offs, that there are family patterns. This family has just been a great, great source of advocacy and strength, and they've gone out and shared it with the rest of the community to, you know, go find care. If you're not getting care where you are, don't give up. I think sometimes families feel defeated if their initial doctor tells them that there's nothing wrong, and I think they're just a great example of just keep pushing until you find someone that listens to you.
Erin: Yeah. And I can see how this transitional care model really facilitates that kind of open dialogue with patients. So how else do you see this transitional care model and all the benefits it provides really impacting care for Chiari patients in the future?
Dr. Greenfield: We're getting more and more slick surgically, minimally invasive, and, um, leaving smaller scars and getting patients out of the hospital faster. But I think at its root, all medicine comes back to diagnostics and, and trying to figure out what we can do for the patient. So there, there are hundreds and hundreds of amazing doctors here, but, like, our lives are incredibly difficult to coordinate. And when we see, you know, 20 patients in a day and need to, like, say, "Well, that patient needs those three resources," how do you ensure that that happens? The way you do that is to create this coordinated care model. I mean, they should all be seen single day with all the testing done and leave with a, a, a coordination of care plan from all the teams involved. So my vision for Chiari is that. You know, that's not different than it should be for epilepsy or for hydrocephalus or for any number of important neurologic diseases that we take care of. But for Chiari, it's been illuminating to see how pushing the envelope has allowed us to, you know, improve the care by taking what was once viewed as a purely, you know, yes or no decision-making for surgery and kind of expanding the realms of, of indications for which we would consider surgery. And listening to the patients to tell us what they're being impacted by has just created this vision for, "Well, if this can work for Chiari, it could work for lots of other things too."
Erin: Let's hope so. Yeah. Well, Dr. Greenfield, it has been such a joy to chat with you today. I have loved hearing about your work and your vision, and I really appreciate you taking the time.
Dr. Greenfield: It's been my pleasure. It was really great getting to know you to talk about these programs.
Erin: Thanks so much to Dr. Jeffrey Greenfield for speaking with me today about his work to pioneer this unique transitional care model and improve outcomes for patients with Chiari malformation. I'm Erin Welsh. Advances in Care is a production of NewYork-Presbyterian Hospital. As a reminder, the views shared on this podcast solely reflect the expertise and experience of our guests. To listen to more episodes of Advances in Care, be sure to follow and subscribe on Apple Podcasts, Spotify, or wherever you get your podcasts. And to learn more about the latest medical innovations from the pioneering physicians at NewYork-Presbyterian, go to nyp.org/advances.
