
Leading-Edge Spina Bifida Care
In this episode, leaders from the multidisciplinary Spina Bifida Center at Lurie Children's discuss their innovative approaches to spina bifida care in the areas of fetal surgery, neurosurgery, orthopedics and urology. Learn about the latest advancements from the center, including a new orthopedic classification system that’s shaping the future of spina bifida treatment.
"We try to stress for every family that we want every child to be their best. To be out and thriving and playing and interacting. Being able to lead productive lives ... and that takes all of us working together."
Robin Bowman, MD
Director, Multidisciplinary Spina Bifida Center
Co-Director, Fetal Neurosurgery, The Chicago Institute for Fetal Health
“We get to know these families from the very beginning. And we can lay out what to expect from our team over a lifetime.”
Elizabeth Yerkes, MD
Director of Neurourology and co-director of Reconstructive Pediatric Urology and the Division of Urology
"Our goal is to maximize the functional ability of each child with spina bifida and maintain their functional goals into adulthood."
Jill Larson, MD
Attending pediatric orthopedic surgeon
Show Notes
- Lurie Children’s specialists in neurosurgery, orthopedics, and urology work in a multidisciplinary approach with families to treat spina bifida and focus on maintaining or improving the child's function throughout their life.
- One area of expertise begins before a child is born, through in utero, spina bifida repair surgery. This procedure involves closing the baby's spine before birth, reducing the severity of spina bifida symptoms, particularly in improving leg strength and lessening the need for treatment of hydrocephalus.
- Dr. Bowman explains how Lurie Children’s has advanced from traditional open fetal surgery to fetoscopic surgery, which minimizes the impact on the mother's uterus. This technique allows for potential vaginal delivery in future pregnancies, highlighting a significant improvement in maternal care.
- The orthopedic team's primary role is to prevent or correct deformities that might hinder independent mobility in patients with spina bifida.
- Annual assessments of muscle strength, nerve function, joint range of motion, and spinal deformity are essential for patients. Initial evaluations begin in infancy, while monitoring continues as the child grows.
- Dr. Larson discusses new classification for spina bifida which is simple to understand and can serve as a functional prognostic guide to help facilitate communication among healthcare professionals. It also serves as an excellent research tool to assess the functional impact of therapeutic and surgical interventions in the spina bifida patient population.
- Dr. Yerkes explains the important role that urology plays in the care of children with spina bifida through their lifetimes, focusing on kidney preservation and achieving continence if that is what a patient and family desires. She discusses individualized continence programs, urodynamic testing, and the importance of self-management for patients as they transition into adolescence and adulthood.
- Dr. Bowman says the future of spina bifida care is bright, with ongoing innovations in fetal surgery and interdisciplinary collaboration. She says the multidisciplinary team at Lurie Children’s is dedicated to lessening the burden of spina bifida and supporting each child’s growth and independence.
Transcript
[00:00:00] Erin Spain, MS: This is Precision Perspectives on Children's Surgery from Ann & Robert H. Lurie Children's Hospital of Chicago. I'm your host, Erin Spain. On this podcast, we introduce you to surgeons at one of the country's most renowned children's hospitals to find out how they are transforming pediatric medicine. The Multidisciplinary Spinal Bifida Center at Lurie Children's provides compassionate, comprehensive care for children with spina bifida and related conditions. The Center has a long history of excellence. And was one of the first multidisciplinary clinics for spina bifida in the country. The team here continues to lead the field. They recently developed a new orthopedic classification system to better assess the prognosis and management of these patients. One of the many contributions to the field aimed to have a positive impact on patient's care. Today, we're going to discuss this recent work and other innovative ways this team is providing care to patients with spina bifida through fetal surgery and neurosurgery, orthopedics, and urology. We're going to speak with three leaders from the Center, Dr. Robin Bowman, Dr. Elizabeth Yerkes and Dr. Jill Larson. Dr. Bowman is the director of the Multidisciplinary Spinal Bifida Center and co-director of Fetal Neurosurgery for the Chicago Institute for Fetal Health. Dr. Yerkes is the director of Neurourology and co-director of Reconstructive Pediatric Urology and the division of Urology. Dr. Larson is an orthopedic surgeon at Lurie Children's with a focus in the management of neuromuscular hip and spine pathologies. Welcome.
[00:01:47] Dr. Robin Bowman: Oh, thanks so much for having us , Erin.
[00:01:49] Erin Spain, MS: Dr. Bowman, spina bifida is the most common permanently disabling birth defect, but every case is different with severity of spina bifida ranging from mild to severe. Can you share the definition of spina bifida and how it's treated at Lurie Children's?
[00:02:06] Dr. Robin Bowman: Sure. Spina bifida is the most complex congenital abnormality of the nervous system that's compatible with long term survival. It's considered a neural tube defect where the bottom end of the spinal cord does not close properly during in utero life and it actually occurs very early in in utero life within the first few weeks of the mother's pregnancy and it's where the spine itself is open and the spinal cord is exposed. It can occur anywhere along the spine, but it usually occurs in the lower back region, which is the area that gives off the nerves to the legs and the bowel and the bladder. And so children with this disability can have weakness or sensory loss in the legs or may have difficulty with bowel or bladder function. It occurs in about 2, 500 babies each year that are born in the United States, and currently it's estimated that somewhere around 160, 000 people are living with spina bifida in the United States. Here in Illinois since 2017 we've been able to offer in utero, meaning surgery when the baby's in the womb, in order to close the back. Although this is, as you'd expect, this surgery carries risk to both the mother and the baby, has been shown in a randomized control trial that it can improve the outcome for the children with open spina bifida. Most notably it can help with the strength in the legs. It can help lessen some of the brain findings and the need for treatment of hydrocephalus, or the extra fluid that can accumulate on the brain. Because this disease can impact the legs and either bowel or bladder function or both, it's very important that the care to the child with spina bifida is multidisciplinary, meaning there's not a single doctor that specializes in all of those areas. And so we all have to work together in conjunction with the parents to give the child the best outcome. Because even though we're now able to offer in utero surgery, not all babies or mothers are candidates for in utero surgery. And even when the child does go through surgery in the womb, It doesn't cure this disease. It lessens the burden of this disease. We try to stress for every family that we want every child to be their best, right? To be out and thriving and playing and interacting. Being able to lead productive lives. And in order to do that we need to manage any disability related to this disease and so that takes all of us working together. The orthopedic surgeons who manage the lower extremities for us to help keep them as functional as possible. The pediatric urologists who manage the bowel and the bladder. And then myself from neurosurgery, who helps take care of the nervous system. And we all work together, working with the parents to make sure that child function is either stable or improving. If we start to see things changing, then it's time to step in and see why things are changing and what we can do to stop things moving in the wrong direction and get them back on a good course toward recovery.
I want to hear more about your expertise with the in utero surgery. Tell me how you were able to begin that program. We started our program in 2017 when our fetal surgeon, Dr. Aimen Shaaban, joined us here at Lurie Children's. I am a pediatric neurosurgeon and I have taken care of children with spina bifida for over 25 years. When you do in utero surgery, this is surgery when the mother's expecting. I have no expertise in taking care of a mom who's expecting. I have the expertise in taking care of the baby. And we really could not expand into the area of fetal surgery until Dr. Shaaban Joined us in 2017 as fetal surgery and caring for the mother who has gone through fetal surgery along with our maternal fetal medicine colleagues from Northwestern, we have been able to offer fetal surgery. And then in 2020, we were able to transition into fetoscopic surgery. Initially, the surgery was similar to the way that it was done in the randomized controlled trial, where in order to approach the baby, we would open the uterus, and close the baby's back and then Dr. Shaaban would close the mother's uterus. That leaves an imprint on the mother's uterus as that scar tissue never quite heals the same and the mother for that pregnancy and all subsequent pregnancies must go through cesarean sections, can never go through labor again. In order to improve the care for the mother, lessen the burden on that uterus we moved toward fetoscopic surgery where we put in three little working ports, 10 French, 10 millimeter ports into the uterus and then using fetoscopic instruments close the baby's back in that manner. And in doing so then as long as from an obstetrical perspective these mothers and babies are able to go through a vaginal delivery.
[00:07:47] Erin Spain, MS: So exciting the progress that has been made in such a short time. Can you tell me a little bit about what you could see coming next down the pike when it comes to the in utero and fetal surgeries?
[00:07:58] Dr. Robin Bowman: Yeah, we're continuing to advance our closures of the back and working with other colleagues to try to come up with some improvement in our instrumentation. Right now there's no true fetal surgery instruments that are out there on the market. And so really helping to move it forward by improving the instruments. We also want to improve our way of teaching the next generation. I have a very basic model which I trained myself on but our hope is to work with some other investigators to establish an improved training model so as to train our residents and fellows in the next generation as to how to do these surgeries to the best of our ability and safely for these mothers and babies.
[00:08:47] Erin Spain, MS: And as you mentioned, this doesn't cure the condition, but it does offer a lot of hope and a lot of improvement. Can you tell me a little bit about what you've seen personally in some of your patients who've undergone the surgery and some of the success that they've had?
[00:09:00] Dr. Robin Bowman: Just as was shown in the randomized control trial, which was called the MOMS trial. We have definitely seen an improvement in the strength of the legs and sensation improved based upon what we would have anticipated that child's legs how they would have functioned based upon where the opening was had that child gone through postnatal closure. We've also seen an improvement or a lessen for a need for treatment of hydrocephalus or the extra fluid in the brain. And then a majority of children that go through in utero surgery will have an improvement in what we call the hindbrain herniation, which is where the brain tissue in the back tends to sink down into the upper neck region in little babies that have open spina bifida. And after you close their back in utero, invariably for almost all of the children, that brain tissue then is back in the normal position, or we always say floats back up into the normal position.
[00:10:05] Erin Spain, MS: And then as you mentioned, once these children are born, there's a whole multidisciplinary team that comes together to provide care. Can you just talk about how everybody works together? How do these specialists really know the patient and how do you all talk to each other and work together to improve the quality of life of your patients?
[00:10:23] Dr. Robin Bowman: Really the best care you can give a child with spina bifida is multidisciplinary care that is patient, family centered, focused. We're partners with the families and ultimately the child and we want to early on establish the child's, what we say, their baseline function. This disease some of our moms refer to it as the snowflake disease. Every child is slightly different and that's very true. And we want to establish what the child's baseline function is and then our goal throughout childhood and a lifetime is to maintain that function or to improve upon it with therapies and activities and at times it takes some surgical intervention, but we want to maintain or improve upon that function. And that really takes all of us working together in conjunction with the parents. Because, again, there's not a single doctor that is an expert in all areas of medicine that these children require.
[00:11:28] Erin Spain, MS: Let's talk with the two of these specialists. We'll start with Dr. Jill Larson. As an orthopedic surgeon at the Center, you want the best functional outcomes for patients with spina bifida, many of whom experience orthopedic problems and muscle skeletal deformities. Tell me about some of the surgical procedures and techniques you use to help these patients optimize mobility, function, and independence.
[00:11:52] Dr. Jill Larson: Well, again, thanks so much for the opportunity to be here. Our role as an orthopedics team is to prevent or correct deformities that may limit a patient from independent walking or mobilizing with assistive devices. Our orthopedic team monitors for scoliosis, also known as curve of the spine, and lower extremity deformities that may be a result of a change in neurologic status, such as a tethered cord or shunt malfunction. Patients need to be assessed on an annual basis so our team can identify any changes in muscle strength or nerve function, assess joint range of motion, or monitor for progression of spinal deformity or worsening skin integrity. Ultimately, our goal is to maximize the functional ability of each child with spina bifida and maintain their functional goals into adulthood.
[00:12:42] Erin Spain, MS: You meet with these families pretty early on. Tell me about those initial conversations and what types of things are you telling the parents right away in those early days?
[00:12:52] Dr. Jill Larson: So I'm usually meeting the family for the first time when they're in the neonatal intensive care unit. And so there's a lot of emotion from the family, from the team taking care of the child at that time. And so there's a lot going on. And so what I really try to emphasize is that I'm here for moral support and guidance along the way. And from a physical standpoint, I'm just trying to help the family take care of their baby, to feed the baby, to help the baby grow and develop. And then we kind of deal with some of the functional limitations with spina bifida as the baby grows and develops, but really in infancy, it's about doing normal baby things, loving a baby, feeding a baby, just meeting basic developmental milestones.
[00:13:37] Erin Spain, MS: You were a coauthor of a paper alongside other Lurie Children's investigators that proposed a new orthopedic classification system specifically for spina bifida. Tell me about the system and why it's needed.
[00:13:50] Dr. Jill Larson: So in spina bifida, several classification systems have previously been published. And our present manuscript proposes a new functional classification system with the foundational hallmark of the manual muscle testing anchoring the classification system. And this objective measurement of the manual muscle test is combined with the functional mobility scale and the use of assistive devices in the classroom. In each patient to provide a more accurate way to describe the functional status in this patient population. We believe that this new classification is simple to understand and it can serve as a functional prognostic guide to help facilitate communication among healthcare professionals. It also serves as an excellent research tool to assess for the functional impact of therapeutic and surgical interventions in the spina bifida patient population.
[00:14:43] Erin Spain, MS: So these little progressions that you're able to make as a community of researchers and bringing more information, you said you're going to make this easier and simplify it a little bit. Why is this so important to help really push the field forward?
[00:14:56] Dr. Jill Larson: So here at Lurie Children's, we have the opportunity to have world class physicians who see a large population portion of the spina bifida population across the country. But that doesn't happen in more rural or smaller areas in the country that don't have access to as many healthcare providers that have this level of knowledge. And so we want to be able to disseminate this knowledge and this education to providers, not just in the Chicagoland area, but across the country and even abroad to help get these patients the care that they need and provide a language of support both for families and healthcare providers to make this patient population as functionally independent as possible. And I think that this new classification system provides a foundation of not only communication and classification, but hopefully also demonstrating prognostic value for functional independence in the future.
[00:15:49] Erin Spain, MS: So is the system being implemented right now at other places around the country? What can you tell me about that update?
[00:15:55] Dr. Jill Larson: The classification system was first developed in about 2020/2021, and we've spoken on this classification system at national meetings across the nation, and I think we're getting the word out more at both educational talks as well as Grand Rounds talks across the country. And then our publication is getting a lot of views widely both nationally and internationally. I think it's becoming more of an accepted classification system and we'll continue to speak on it and educate others so that it can become more of a tool that healthcare providers can use.
[00:16:28] Erin Spain, MS: Now we're going to shift gears a little bit and talk specifically about urology's role in the care of children with spina bifida, with Dr. Elizabeth Yerkes. Now your focus is related to continent self-management and renal care of your patients. Tell me about this and the role urology plays in the care of these children.
[00:16:47] Dr. Elizabeth Yerkes: Yeah, so we get to know these families from the very beginning . And we can sort of lay out what to expect from our team over a lifetime. And of course, you know, there may be some bumps along the way that aren't apparent from that initial communication, but that's what we're there for, to work through those things. The top three things that we're looking to do for these kids are to manage the bladder in such a way that we preserve kidney function. Obviously, the kidneys provide a very vital function for us to filter our blood, that's what makes the urine. We need to manage the urine appropriately on the other end so that we're not harming the kidneys. So, managing the pressure in the bladder, doing everything we can to prevent urinary tract infections that could go to the kidney. As the child ages, then we start focusing on continence and we have the very glamorous job of taking care of both urine and stool way more glamorous than the other specialties in the clinic, I think. And it's obviously a very intimate topic or process that we're helping these patients and families with, so trying to negotiate that, and definitely it's not one size fits all. Each child may need something different. Each family may be prepared or committed to something different. They may have a different threshold for beginning a continence program. But the number one thing throughout that is that we're trying to manage the bladder to protect the kidneys . And in some cases that results in continence and in some cases they may be incontinent and at risk of kidney damage. So each child is approached separately. There's specialized testing called urodynamics. So these are the three main things that we do that essentially profile the bladder. They let us know how much does the bladder hold what pressures are occurring in the bladder that may put the kidneys at risk, what is the reason that we're not able to achieve continence, and how can we move toward that if the family is desirous of moving toward a continence program. So over time we'll be following these children from infancy through to teenage years and into adulthood and we're monitoring the health of the kidneys through ultrasound and blood work over time, but periodically will obtain specialized tests of bladder function called urodynamics . And this allows us to essentially profile the bladder. It'll let us know is the bladder storing at safe pressures, is the bladder generating pressures that are potentially harmful to the kidneys over time or maybe even harmful to the bladder itself. And it also helps us determine what the source of incontinence might be so that we can tailor the medical program for the patient to help them achieve both, a safe situation for the kidneys, but also to move toward continence. After we've worked with the family on continence, the additional goals, the reason that we're trying to do these things is so that the child can participate in classes at school, participate in afterschool activities, perhaps participate in adaptive sports, which a lot of our patients do. It's very exciting to hear about. They can do that because they're taking part in a continence program, and that allows them to be kids, to do all the things they want to do. And for our teens and young adults, it allows them to participate in the community as well, perhaps have a job. Participate in sports, have relationships, and all of our goals are to help them do what they would like to do to the best of their abilities. And in order to do that, in order to be out of the home and out of the school environment and care for themselves, they have to achieve self confidence with their bladder and bowel program, whatever that might be. Maybe they don't need much, maybe they just need to be on a schedule to go to the bathroom or perhaps they need to catheterize or take medication or do an enema program. Whatever they need, we're there to support them through that. And some days will be great days and some days will be less good and we always want to hear from families to help them make every day a better day .
[00:20:05] Erin Spain, MS: I want to dig into that a little bit more. You know, patients and families might have different goals when it comes to managing continence. So what role do you play in helping these families navigate what might be the best choice for their child?
[00:20:17] Dr. Elizabeth Yerkes: You're right it's definitely not a one size fits all and every family doesn't have the same goals and every patient doesn't have the same goals. And so we shouldn't presume to know what they would want to have. Again, as long as we're keeping the kidneys safe, continence is really more of a social concern than a medical concern. So the families can have a little latitude there to choose what feels right. Making families aware that opportunities exist for continents, first of all , and then, you know, there's a stepwise approach of invasiveness and commitment that we work through with the families. And we can look at the aerodynamics and say this, this would likely be what would be the appropriate first step for your child. We might need to do something additional in the future. We don't know until we try the first step. And then just trying to get a sense of the family and what their resources are, what their day to day is like. Do they have you know, 45 minutes to an hour for a bowel program in the evening. Is the benefit of continence worth that time for them? Or do they have a precise time every day that they can commit? So learning about the family and not only their priorities, but also their resources is really important .
[00:21:18] Erin Spain, MS: Despite all your best efforts, sometimes surgical reconstruction for continence or kidney protection may be required. Tell me how Lurie Children's stands apart with this type of surgery.
[00:21:29] Dr. Elizabeth Yerkes: I think it is counseling the thoughtful consideration of what each child may actually need and benefit from. There's a lot of surgery we can, from a urologic standpoint, a lot of procedures that can help achieve continence of urine and stool and they're very effective procedures, but they also come with some baggage. And so, we want to make sure that the families have a really clear understanding of what the options are. What the immediate and longer term risks are and they may not have a high long term risk of having either some sort of nuisance complications that can happen over time, but they also have a small risk of having a serious complication, or they have the potential of not achieving what we were setting out to achieve. And so I think families have to be able to go into these decisions eyes wide open, very well informed by the person who's going to perform the surgery. And I think what our group does is in addition to the careful counseling is being very active in the follow up. I sort of view this as a marriage with these families. Eventually I'll retire and step out of the marriage. But It's a commitment that we're making to each other. It's something that I am doing for and with them to help them achieve their goals.
[00:22:32] Erin Spain, MS: It's true. This team is really helping patients achieve their goals. Dr. Bowman, can you tell me a little bit about how the work happening inside of the clinic influences the lives of your patients outside of the clinic?
[00:22:45] Dr. Robin Bowman: Our goal is to address all of these medical complexities, but at the same time remember that these are children, and these are children that at times can have either mild or significant disabilities, but we want every child to be out there being a kiddo achieving, learning to be independent, to grow independent from mom and dad, so that long term they can lead a life that's productive and fulfilled. And so our goal is to help these kids gain independence. We want to make sure that they remain healthy and fit. And at the same time, encourage them to be independent And so, our team we all help out with our Y M C A Camp Independence and encourage the kids to come and through camping learn that indeed They can be independent of mom and dad and have fun and get to know other children, right? Have a social network of friends . Because at times this is a life that is different than many of their peers. And to take care of themself, to take good care of themselves requires more activity. It requires a higher burden of care. And at times that can feel very lonely. And it can feel like they're the only ones that have to do certain routines for their bladder or certain routines for their bowel or wear braces. And so It's terrific because we get to know all of these families and all of these great kids and we love when they get to know each other through different sporting activities, through their wheelchair basketball, or through their sled hockey, or when they get to know each other up at camp because they're just a fantastic group of kids and I love that they're able to form these social networks and get to know each other and support each other through the years.
[00:24:34] Erin Spain, MS: As we wrap up today, what do you want to leave listeners with when we think about the future of care for children with spina bifida? What do you want our listeners to know?
[00:24:43] Dr. Robin Bowman: The future is very bright. Spina bifida is not a disease that we have a cure for, but there is definitely a momentum of working toward lessening the burden of this disease. This disease is changing over time, and by working together, and it really is a team effort. It's a team effort with the healthcare professionals, with the families, and ultimately the patient, that our goal is to work together so that we can lessen the burden of this disease and support each child so that they can be the very best that they can be as they grow and develop.
[00:25:22] Erin Spain, MS: Thank you so much, Dr. Bowman, Dr. Larson and Dr. Yerkes for coming on the show. We appreciate all of your insight today.
[00:25:30] Dr. Robin Bowman: Thank you so much for inviting us.
[00:25:32] Erin Spain, MS: For more information, including how to make a referral or an appointment, visit luriechildrens.org.
Other Episodes

Advancing Congenital Heart Disease Care with Dr. Kiona Allen
In this episode, Dr. Kiona Allen discusses key components of a supportive, long-term approach for children with congestive heart (CDH) disease.
Read More

Helping Families Navigate Drug-Resistant Pediatric Epilepsy with Dr. Sandi Lam
In this episode, Dr. Sandi Lam, Division Head of Neurosurgery at Lurie Children’s, talks about her work to help pediatric patients with Lennox-Gastaut syndrome and their families manage this condition by investigating the outcomes that matter most to patients and their families.
Read More

Fertility Preservation Innovations for Children with Monica Laronda, PhD
In this episode, Dr. Monica Laronda, an expert in the field of reproductive biology, shares many new projects she is leading alongside surgeons in Lurie Children’s Fertility & Hormone Preservation & Restoration Program.
Read More